Prominent Component Research Articles

Thymoquinone and Melatonin Boost Both Sirt1 and Nrf2 Activities in a Complementary Way – A Strategy for Controlling Oxidative Stress and Inflammation

The oil of black cumin seed (Nigella sativa) has been employed therapeutically in Asian traditional medicine, and rodent studies with it or its most prominent and intriguing component, thymoquinone, suggest that it may indeed have the potential to provide benefit in a wide range of pathologies. There is reason to suspect that thymoquinone’s versatile utility may reflect, in large part, its ability to boost the activity of the sirtuin 1 (Sirt1) deacetylase while concurrently activating the Nrf2 transcription factor. Increased Sirt1 activity promotes autophagy, mitochondrial biogenesis, and FOXO-mediated expression of antioxidant enzymes, while suppressing NF-kappaB-mediated inflammatory signaling; Nrf2 promotes expression of a wide range of antioxidant enzymes, as well as the enzyme rate limiting for glutathione synthesis. Thymoquinone is a substrate for NAD(P)H quinone oxidoreductase 1 (NQO1); this reaction gives rise to NAD+ - obligate substrate for Sirt1 activity – while generating thymohydroquinone, a potent scavenging antioxidant. Thymoquinone, which is electrophilic, promotes Nrf2 activation through covalent reaction with its functional inhibitor Keap1. We suggest that supplemental melatonin may be an ideal complement to thymoquinone, as it can promote increased expression of both Sirt1and Nrf2 – likely via activation of the “clock” transcription factor Bmal1 – whereas thymoquinone boosts the biological activities of the pre-formed proteins.

Uncommon Pathological Presentations of Mature Cystic Teratoma: A Case Series

Mature cystic teratomas are benign tumours of the ovary but rarely certain unusual gross and microscopic findings might complicate their diagnosis or may have an impact on their clinical outcome. Here, we are presenting a case series of five unusual pathological presentations of mature cystic teratomas of ovary. Out of five cases, three cases were of collision tumours, in which combination of mature cystic teratoma is seen with mucinous cystadenoma in two cases, and with serous cystadenoma in one case. True collision tumours are defined as histologically distinct neoplasms in the same tissue or organ without any histologic admixture or intermediate cell population zone between two components. These tumours are located in various organs but ovarian location is rare. Mature cystic teratoma is the most common component of collision combinations in the ovary. All classes of ovarian tumours, benign, borderline and malignant may collide and therefore, clinical outcomesin collision tumours depend on individual tumour characteristics. Fourth case was of a mature cystic teratoma associated with a distinct hemangiomatous component. Mature cystic teratoma associated with prominent hemangiomatous component is a very rare finding with only few case reports in the literature and it should be differentiated from true ovarian hemangioma, lymphangioma and angiosarcoma. Fifth case was of bilateral mature cystic teratomas associated with a well-defined lipomatous lesion in right sided mature cystic teratoma mimicking lipoma. Lipomatous ovarian lesions are very rare and mature cystic teratoma with prominent adipocytic proliferation is also an exceedingly uncommon finding and it should be differentiated from true ovarian lipoma and other atypical lipomatous proliferations. Most of these tumours remain clinically and radiologically unrecognized, therefore their histological recognition is essential for adequate patient management.

Follow-up for a Preterm Infant with Beckwith-Wiedemann Syndrome.

Follow-up for a Preterm Infant with Beckwith-Wiedemann Syndrome.

Dead Space Breathing in Patients with Malignancies: Determination by Cardiopulmonary Exercise Testing

Rationale: Patients with cancer commonly experience dyspnea originating from ventilatory, circulatory and musculoskeletal sources, and dyspnea is best determined by cardiopulmonary exercise testing (CPET). Objectives: In this retrospective pilot study, we evaluated patients with hematologic and solid malignancies by CPET to determine the primary source of their dyspnea. Methods: Subjects were exercised on a cycle ergometer with increasing workloads. Minute ventilation, heart rate, breathing reserve, oxygen uptake (V’O2), O2-pulse, ventilatory equivalents for carbon dioxide and oxygen (V’E/V’CO2 and V’E/V’O2, respectively) were measured at baseline and peak exercise. The slope and intercept for V’E/V’CO2 was computed for all subjects. Peak V’O2 4% predicted indicated a circulatory or ventilatory limitation. Results: Complete clinical and physiological data were available for 36 patients (M/F 20/16); 32 (89%) exhibited ventilatory or circulatory limitation as shown by a reduced peak V’O2 and 10 subjects with normal physiologic data. The largest cohort comprised the pulmonary vascular group (n = 18) whose mean ± SD peak V’O2 was 61% ± 17% predicted. There were close associations between V’O2 and spirometric values. Peak V’E/V’O2 and V’E/V’CO2 were highest in the circulatory and ventilatory cohorts, consistent with increase in dead space breathing. The intercept of the V’E-V’CO2 relationship was lowest in patients with cardiovascular impairment. Conclusion: Dyspneic patients with malignancies exhibit dead space breathing, many exhibiting a circulatory source for exercise limitation with a prominent pulmonary vascular component. Potential factors include effects of chemo- and radiation therapy on cardiac function and pulmonary vascular endothelium.

Investigating change across time in prevalence or association: the challenges of cross-study comparative research and possible solutions

Cross-study research initiatives to understand change across time are an increasingly prominent component of social and health sciences, yet they present considerable practical, analytical and conceptual challenges. First, we discuss the key challenges to comparative research as a basis for detecting societal change, as well as possible solutions. We focus on studies which investigate changes across time in outcome occurrence or the magnitude and/or direction of associations. We discuss the use and importance of such research, study inclusion, sources of bias and mitigation, and interpretation. Second, we propose a structured framework (a checklist) that is intended to provide guidance for future authors and reviewers. Third, we outline a new open-access teaching resource that offers detailed instruction and reusable analytical syntax to guide newcomers on techniques for conducting comparative analysis and data visualisation (in both R and Stata formats).

Abstract- Poster

Background:Irritable bowel syndrome (IBS) is a chronic psycho-physiological disorder, considered to be the most common functional gastrointestinal disorder. About 50–90% of IBS patients have associated psychiatric co-morbidity.It is also well-established that patients with IBS have higher levels of anxiety and depression compared to controls.Aim-To contribute in the literature of relationship between IBS and psychiatric diseases.Methods:A 50 y/o female patient was brought to psychiatry opd with chief complaints of:- · Multiple episodes of constipation, with mild abdominal pain· Repetitive urges to evacuate manually, by fingers, if not done patient becoming restless, around 30-40 times a day.· Restlessness when not allowed to evacuate manually. Patient was diagnosed as IBS-C 8 years back, and since 3 years was having the above mentioned symptoms.The patient was treated on the lines of OCD spectrum and anxiety. Supportive counseling was done and patient was prescribed T. Escitalopram 10mg and T. Clonazepam as and when required.Results-There was significant improvement in urge to evacuate manually within 2-3 months of treatment in both OCD, anxiety and IBS.Conclusion:IBS pathophysiology is not well understood; however, it is hypothesized to be a disorder of the “gut-microbiota-brain axis”. Dysfunction in some of these pathways have been implicated in IBS and in psychiatric disorders. Anxiety is prominent component of other disorders like OCD. While several lines of evidence suggest that the gut-brain axis might be relevant to OCD, the relationship between OCD and IBS is less exploredAcknowledgementsDr. Minakshi Parikh(Professor and Head, Department of Psychiatry, B.J. Medical College and Civil Hospital, Ahmedabad)Dr. Sarandha Srivastava(Assistant Professor, Department of Psychiatry, B.J. Medical College and Civil Hospital, Ahmedabad)

Estimating cation exchange capacity and clay content from agricultural soil testing data

Clay content and the ability to reversibly retain cations affect many essential chemical and physical properties of soil, such as pH buffering and carbon sequestration. Cation exchange capacity (CEC) and base saturation are also commonly used as criteria in soil classification. However, determination of CEC and particle-size distribution is laborious and not included in routine soil testing. In this study, pedotransfer functions including soil test cations (STCat; Ca2+ + Mg2+ + K+), pH and soil organic carbon (SOC, %) as explanatory variables were developed for estimating CEC, titratable acidity (TA; H+ + Al3+) and clay content (clay, %). In addition, reference values for potential CEC and its components were determined for Finnish mineral and organic soils. The mean of potential CEC extracted by 1 M ammonium acetate at pH 7.0 ranged from 14 (range 6.4−25) in coarse soils to 33 (21−45) cmol(+) kg-1 in heavy clay soils, and from 42 (24−82) in mull soils to 77 (25−138) cmol(+) kg-1 in peat soils. The average CEC of clay and SOC were 27 and 160 cmol(+) kg-1, respectively. Titratable acidity occupied 53% and around 40% of the CEC sites in organic and mineral soils, respectively, evidencing that it is a prominent component of the potential CEC in these predominantly acidic soils. STCat, pH and SOC explained 96% of the variation in potential CEC. STCat and pH can be used in estimating the clay content especially for soils containing over 30% clay. In coarse textured soils, in contrast, SOC hampers the STCat based estimation of clay content.

Increased cognitive load during pseudoword processing

Pseudowords (words without semantic meaning) are often used as a control condition in linguistic cognitive experiments, with the expectation that such words, unlike real words, do not activate higher cognitive processes in the brain. However, other theories assume that pseudowords are perceived as new words, leading to an even higher cognitive response. The aim of this study was to investigate the cognitive load of processing a pseudoword by observing event-related potentials in a 3-stimulus oddball paradigm using real target words, real non-target words, and pseudowords as oddball stimuli. The results show a clear task-related P3b triggered by target words, but also a prominent P600 component triggered by pseudowords, indicating difficulty in the classification task due to unknown words. Surprisingly, N400 was decreased for pseudowords compared to target and non-target words at the locations where P3b and P600 were observed, suggesting that task-related effects might inhibit other aspects of cognitive processing. These results could lead to better understanding of the components that may overlap temporally and topographically, and to the more precise control of different cognitive generators involved in event-related potential experiments in pseudowords.

Counting trees in a subtropical mega city using the instance segmentation method

Counting the trees in a given area or city is meaningful when making decisions for government policies and administration, including the international afforestation effort (i.e., the Trillion Trees Campaign). Determining individual trees on a large scale poses significant challenges, especially in subtropical and tropical areas, because of their diverse crown characteristics. Using very high-spatial-resolution images, we can see the tree crowns clearly. In this study, we counted the population number of trees in the subtropical mega city of Guangzhou, and we used an end-to-end tree-counting deep-learning framework in the regional-scale tree detection by delineating each tree crown. It is a simple framework in which individual trees can be detected directly without manual operation. We used the cascade mask regions with convolutional neural networks (CMask R-CNN) as the backbone and added three types of attention modules to build the derivatives of the CMask R-CNN. The experimental results showed that the CMask R-CNN performed the best among all of the methods, and more than 112 million individual trees with crown sizes of large than 1 m2 were detected. The experimental assessment indicated that the accuracy was 88.32% in terms of the R2 value and 82.56% in terms of the F1-score. This study not only revealed the number of trees, but it also provided the tree density at different scales, which is a prominent component of the ecosystem structure. We also analyzed the tree density at the 30 m and 1000 m scales. The experimental results showed that Guangzhou has a high canopy cover with a tree density of 150 trees per hectare. For the entire city, the tree density is highest in the northern area of Guangzhou, followed by the central part. From the central-western part to the central-eastern part, the tree density increases. The lowest tree density is located in the southern part of Guangzhou near the Pearl River estuary, which is a basic farmland conservation area. It is notable that the tree density of the urban land is about 15 trees per 900 m2, indicating a good living environment. The method developed in this study provides a flexible means of large-scale tree counting without manual operation based on very high-spatial-resolution images.

Assimilating problematic life script themes in clinical supervision: The case of Sarah

AbstractObjectivesLife script is a transactional analysis concept describing a pattern of human experience, interaction and meaning making developed in childhood that can be activated in adulthood, sometimes creating problems. Problematic life script themes can impact a therapist's experience and interaction during clinical work and interfere with therapeutic effectiveness. This study used the comparative script system theory to assess how one therapist's problematic life script themes were manifested in supervision, how they were addressed, and whether addressing them advanced assimilation of the problematic material.MethodSeven consecutive monthly supervision sessions with a 50‐year‐old, highly experienced female therapist in private practice (pseudonym ‘Sarah’) were analysed by a team of five investigators using a qualitative theory‐building approach. The assimilation of problematic experiences sequence (APES) was used to track changes in Sarah's life script themes.ResultsProblematic life script themes were evident in each supervision session. Progress though the APES varied across themes and six different clients discussed during the sessions.ConclusionsThe supervisee's life script themes emerged prominently in supervision session content, and most were successfully navigated. The observations supported the theoretical suggestion that assimilation of supervisee unprocessed life script material may be a prominent component of routine professional supervision.

Equivalent Input Disturbance-Based Control Design for Three Phase Dual-Stage Grid-Tied Photovoltaic System Considering Dead Time Effect

Grid-tied inverter is the prominent component of the three-phase dual-stage photovoltaic (PV) grid-tied power generation system. However, the disturbances caused by dead time effect will pose the reduction of grid-tied current quality and even cause the imbalance of inverter itself or other circuit devices. In this paper, a current control strategy is proposed to damp dead time effect for the three-phase dual-stage PV grid-tied inverter system, and its design, stability analysis, and implementation are carried out. First, the inverter model is modified by regarding the dq reference frame coupling terms, uncertainties, and external and internal disturbances as an unknown lumped disturbance. Then, a current control scheme based on compensation of equivalent input disturbance is introduced, and it estimates and compensates the unknown lumped disturbance, which effectively realizes the inverter model decoupling and comprehensive disturbance rejection. Last, simulation results demonstrate the effectiveness and superiority of the proposed current controller.

Technical aspects of the reconstruction of historic gardens

Abstract Built elements and structures are a prominent component of our historic gardens, both in terms of function and artistic composition and garden scenery. The surveys of historic garden structures are important research tasks, which also underpins and validates restoration work. In most cases, the neglected state of historic gardens and sites and the unavailable archival materials do not allow an authentic restoration of historic gardens to their original state. Nevertheless, there is a real need to reconstruct our historic gardens, based not only on historical authenticity but also on a systematic reinterpretation of the relationship between society and landscape. The objective of this article is to present a general methodology for renewal of historic gardens through examples of specific garden reconstructions. The case studies are the authors' own design works, which demonstrate the application of different design approaches, highlighting details of the reconstruction of specific built garden elements.

Immunohistochemical comparison of p63 and p40 in head and neck Spindle Cell Carcinoma

BackgroundSpindle Cell Carcinoma (SpCC) is a rare type of squamous cell carcinoma (SCC) with prominent malignant spindle cell component. This unique biphasic feature on histopathological examination makes its diagnosis problematic. Detection of p63 antigens in SpCC can be helpful however its expression in variousb proliferating soft tissue lesions demands for better marker. MethodsIn this study, histopathologically diagnosed SpCC of head and neck region were considered as cases, and 22 soft tissue sarcomas, reactive lesions and spindle cell lesions of the body were taken as controls. Immunohistochemistry (IHC) was done using Anti-p63 and p40 clone and the results were compared. CK was done for negative cases to prove their epithelial origin. P. value < 0.05 considered statistically significant. ResultsAmong 22 cases of SpCC, 19 cases showed positive immunoreactivity to p63, and 18 cases for p40. IHC of controls showed no immunoreactivity in any of the sarcomas, reactive lesions or spindle cell lesions. The sensitivity of p63 is 86% while that of p40 is 82%. Specificity of both the markers was 100% ConclusionThough p63 is a slightly (4%) more sensitive marker than p40, percentage of cell positivity for p40 is higher compared to p63. Both of these markers are 100% specific for SpCC.

Two new cryptic species of the freshwater fish genus Gobiomorphus (Gobiiformes: Gobioidei: Eleotridae) in New Zealand

ABSTRACT We describe two new species in the genus Gobiomorphus, a radiation of fresh and brackish water gudgeons known from Australia and New Zealand. These species are a prominent component of New Zealand’s freshwater ichthyofauna and most are widely distributed throughout both the North and South Islands. Two of the inland species, G. breviceps and G. basalis, are composed of disjunct northern and southern populations that are distinguishable with molecular data. We examine individuals from across the ranges of both species, identify morphological differences between them, and describe two new species: Gobiomorphus dinae n. sp. (distinct from G. basalis) and Gobiomorphus mataraerore n. sp. (distinct from G. breviceps). Although the species are similar, they vary in dorsal spine count (G. dinae) and pectoral fin ray count (G. mataraerore). We provide mitochondrial COI sequences for each species pair to facilitate identifications by DNA barcoding. These species represent examples of divergence in allopatry, with diagnostic characters arising over the last 2−5 million years in the G. breviceps/G. mataraerore pair, and fewer than 2 million years in the G. basalis/G. dinae pair. We also designate a lectotype for G. basalis (the paralectotype is G. cotidianus) in order to clarify confusion surrounding the original syntypes.

Structural insights into the substrate recognition and catalytic mechanism of a fungal glycoside hydrolase family 81 β-1,3-glucanase

β-1,3-Glucan constitutes a prominent cell wall component being responsible for rigidity and strength of the cell wall structure in filamentous fungi. Glycoside hydrolase (GH) family 81 endo-β-1,3-glucanases which can cleave the long chain of β-1,3-glucans play a major role in fungal cell wall remodeling. Here, we reported the complex structures of a fungal GH family 81 endo-β-1,3-glucanase from Rhizomucor miehei (RmLam81A), revealing the triple-helical β-glucan recognition and hydrolysis patterns. In the crystals, three structured oligosaccharide ligands simultaneously interact with one enzyme molecular via seven glucose residues, and the spatial arrangement of ligands to RmLam81A was almost identical to that of β-1,3-glucan triple-helical structure. RmLam81A performed an inverting catalysis mechanism with Asp475 and Glu557 severing as the general acid and base catalyst, respectively. Furthermore, two hydrophobic patches involving Tyr93, Tyr106, Ile108, Phe619 and Tyr628 alongside the ligand-binding site possibly formed parts of the binding site. A ligand-binding motif, β31-β32, consisting of two key residues (Lys622 and Asp624), involved the recognition of a triple-helical β-glucan. Our results provided a structural basis for the unique β-1,3-glucan recognition pattern and catalytic mechanism of fungal GH family 81 endo-β-1,3-glucanases, which may be helpful in further understanding the diverse physiological functions of β-1,3-glucanases.

Low‐grade epilepsy‐associated neuroepithelial tumours with a prominent oligodendroglioma‐like component: The diagnostic challenges

We searched for recurrent pathological features and molecular alterations in a retrospective series of 72 low-grade epilepsy-associated neuroepithelial tumours (LEATs) with a prominent oligodendroglioma-like component, in order to classify them according to the 2021 World Health Organization (WHO) classification of central nervous system (CNS) tumours. Centralised pathological examination was performed as well as targeted molecular analysis of v-Raf murine sarcoma viral oncogene homologue B (BRAF) and fibroblast growth factor receptor 1 (FGFR1) by multiplexed digital polymerase chain reaction (mdPCR). DNA methylation profiling was performed in cases with sufficient DNA. In cases with no genetic alteration by mdPCR and sufficient material, RNA sequencing was done. We first reclassified our cohort into three groups: ganglioglioma (GG, n = 14), dysembryoplastic neuroepithelial tumours (DNTs, n = 19) and glioneuronal tumours/paediatric-type low-grade glioma (LGG) not otherwise specified (GNT/PLGG NOS, n = 39). mdPCR found an alteration in 38/72 cases. Subsequent RNA sequencing revealed a fusion transcript involving BRAF, FGFR1/2/3 or neurotrophic tyrosine kinase receptor type 2 [NTRK2] in 9/25 cases. DNA methylation profiling found 12/46 cases with a calibrated score ≥0.9. Unsupervised hierarchical clustering revealed two clusters: Cluster 1 was enriched with cases classified as DNT at histology, belonging to the LGG-DNT methylation class (MC), with haematopoietic progenitor cell antigen (CD34) negativity and FGRF1 alterations; Cluster 2 was enriched with cases classified at histology as GG, belonging to the LGG-GG MC MC, with BRAF V600E mutation and CD34 positivity. The tumours reclassified as GNT/PLGG NOS were equally distributed across both clusters. Interestingly, all polymorphous low-grade neuroepithelial tumour of the young belonged to Cluster 2, whereas diffuse LGG mitogen-activated protein kinase (MAPK) pathway-altered were equally distributed among the two clusters. This led us to build an algorithm to classify LEATs with a prominent oligodendroglioma-like component. Integrated histomolecular diagnosis of LEATs with a prominent oligodendroglioma-like component remains challenging. Because these tumours can be split into two major clusters of biological significance, the clinicopathological relevance of the four types recognised by the WHO CNS5 within this spectrum of tumours is questionable.

The transformation of cancer-associated fibroblasts: Current perspectives on the role of TGF-β in CAF mediated tumor progression and therapeutic resistance.

Over the last few years, the Transforming growth factor- β (TGF-β) has been significantly considered as an effective and ubiquitous mediator of cell growth. The cytokine, TGF-β is being increasingly recognized as the most potent inducer of cancer cell initiation, differentiation, migration as well as progression through both the SMAD-dependent and independent pathways. There is growing evidence that supports the role of secretory cytokine TGF-β as a crucial mediator of tumor-stroma crosstalk. Contextually, the CAFs are the prominent component of tumor stroma that helps in tumor progression and onset of chemoresistance. The interplay between the CAFs and the tumor cells through the paracrine signals is facilitated by cytokine TGF-β to induce the malignant progression. Here in this review, we have dissected the most recent advancements in understanding the mechanisms of TGF-β induced CAF activation, their multiple origins, and most importantly their role in conferring chemoresistance. Considering the pivotal role of TGF-β in tumor perogression and associated stemness, it is one the proven clinical targets We have also included the clinical trials going on, targeting the TGF-β and CAFs crosstalk with the tumor cells. Ultimately, we have underscored some of the outstanding issues that must be deciphered with utmost importance to unravel the successful strategies of anti-cancer therapies.

The extreme properties of the nearby hyper-Eddington accreting active galactic nucleus in IRAS 04416+1215

ABSTRACT The physical properties of the accretion flow and of the X-ray emitting plasma, in supermassive black holes accreting at extreme Eddington rates, are still very unclear. Here we present the analysis of simultaneous XMM–Newton and NuSTAR observations of the hyper-Eddington Seyfert 1 galaxy IRAS 04416+1215, carried out in 2020. The main goal of these observations is to investigate the properties of the X-ray corona, as well as the structure of the accretion flow and of the circumnuclear environment, in this regime of extreme accretion. IRAS 04416+1215 has one of the highest Eddington ratio (λEdd ≃ 472) in the local Universe. It shows an interesting spectral shape, very similar to the standard narrow-line Seyfert 1 galaxy’s spectra, with the presence of multiphase absorption structure composed of three phases, whose estimate of the minimum and maximum distances suggests two different interpretations, one consistent with the three X-ray winds being cospatial, and possibly driven by magnetohydrodynamical processes, the other consistent with the multiphase winds being also multiscale. The X-ray spectrum of IRAS 04416+1215 also has a prominent soft excess component and a hard X-ray emission dominated by a reflection component. Moreover, our detailed spectral analysis shows that IRAS 04416+1215 has the lowest coronal temperature measured so far by NuSTAR (kTe = 3–22 keV, depending on the model). This is consistent with a hybrid coronal plasma, in which the primary continuum emission is driven by pair production due to high-energy tail of the energy distribution of non-thermal electrons.

InSAR-based characterization of rock glacier movement in the Uinta Mountains, Utah, USA

Abstract. Rock glaciers are a prominent component of many alpine landscapes and constitute a significant water resource in some arid mountain environments. Here, we employ satellite-based interferometric synthetic aperture radar (InSAR) between 2016 and 2019 to identify and monitor active and transitional rock glaciers in the Uinta Mountains (Utah, USA), an area of ∼3000 km2. We used mean velocity maps to generate an inventory for the Uinta Mountains containing 205 active and transitional rock glaciers. These rock glaciers are 11.9 ha in area on average and located at a mean elevation of 3308 m, where mean annual air temperature is −0.25 ∘C. The mean downslope velocity for the inventory is 1.94 cm yr−1, but individual rock glaciers have velocities ranging from 0.35 to 6.04 cm yr−1. To search for relationships with climatic drivers, we investigated the time-dependent motion of three rock glaciers. We found that rock glacier motion has a significant seasonal component, with rates that are more than 5 times faster during the late summer compared to the rest of the year. Rock glacier velocities also appear to be correlated with the snow water equivalent of the previous winter's snowpack. Our results demonstrate the ability to use satellite InSAR to monitor rock glaciers over large areas and provide insight into the environmental factors that control their kinematics.

Chitotriosidase as a biomarker for gangliosidoses

Elevated serum chitotriosidase (CHITO) is an indication of macrophage activation, and its capacity have been explored as a marker of inflammation in a number of disease states. For over a decade, CHITO plasma levels have been used by clinicians as a biomarker of inflammation in the lysosomal disease, Gaucher disease, including monitoring response to therapies in patients with Gaucher disease type I. Although it is becoming increasingly recognized that inflammation is a prominent component of many lysosomal diseases, the relation of CHITO levels to disease burden has not been well-characterized in the large majority of lysosomal diseases. Moreover, the role of CHITO in lysosomal diseases that affect the central nervous system (CNS) has not been systematically studied. In this study, one hundred and thirty-four specimens of CSF and serum were collected from 34 patients with lysosomal diseases affecting the CNS. This study included patients with GM1-gangliosidosis, GM2-gangliosidosis, mucopolysaccharidoses (MPS), multiple sulfatase deficiency and Gaucher disease. CHITO levels in the CSF were significantly higher in patients with more rapidly progressing severe neurological impairment: GM1-gangliosidosis vs MPS (p < 0.0001); GM2-gangliosidosis vs MPS (p < 0.0001). CHITO levels were higher in patients with the more severe phenotypes compared to milder phenotypes in GM1-gangliosidosis and GM2-gangliosidosis (serum CHITO in GM1-gangliosidosis infantile vs juvenile p = 0.025; CSF CHITO in Tay-Sachs infantile vs Tay-Sachs late-onset p < 0.0001). Moreover, higher CHITO levels in the CSF were significantly associated with lower cognitive test scores in patients with GM1-gangliosidosis, GM2-gangliosidosis, and MPS (p = 1.12*10−5, R2 = 0.72). Patients with infantile GM1-gangliosidosis showed increasing CSF CHITO over time, suggesting that CSF CHITO reflects disease progression and a possible surrogate endpoint for future clinical trials with infantile GM1-gangliosidosis. In summary, these results support the use of CSF CHITO to diagnose between different disease phenotypes and as a valuable tool for monitoring disease progression in patients. These results necessitate the inclusion of CHITO as an exploratory biomarker for clinical trials.