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Local knowledge about natural resources is increasingly crucial in formulating conservation strategies and activities. This study collects data regarding medicinal plant use from Sepahat and Tamiang village residents. A total of 100 participants were included in the study, consisting of 50 participants from the Sepahat and another 50 from the Tamiang. Respondents were selected using the Snowball sampling technique, which involved identifying a key individual, such as local leaders, to initiate recruitment of other respondents. The study's findings indicate that the inhabitants of Sepahat village utilize 36 plant species belonging to 24 families, while Tamiang villagers use 11 plant species belonging to eight families. In Sepahat village, the botanical components used for medication comprise 25% rhizomes, 8% bark, 22% fruit, 3% shoots, 34% leaves, and 8% stems. In contrast, Tamiang comprises 23% rhizomes, 16% bark, 15% fruit, and 46% leaves. The utilization of stems and bark has the potential to pose a threat to sustainability. Among various uses, it has been observed that approximately 33% in Sepahat and 27% in Tamiang can lead to plant mortality. Conservation initiatives have been undertaken for 71% of Sepahat and 67% of the utilized plant species in Taminag. Traditional medicines have been used for generations to treat various health conditions, such as back pain, bleeding, controlling cholesterol, coughs, dengue fever, and diabetes. While current use might not threaten the biosphere reserve, villagers must embrace sustainable harvesting techniques, including selective harvesting, replanting, and establishing community nurseries dedicated to cultivating medicinal plants [ABSTRACT FROM AUTHOR]

The sulfolipid sulfoquinovosyl diacylglycerol (SQDG), produced by plants, algae, and cyanobacteria, constitutes a major sulfur reserve in the biosphere. Microbial breakdown of SQDG is critical for the biological utilization of its sulfur. This commences through release of the parent sugar, sulfoquinovose (SQ), catalyzed by sulfoquinovosidases (SQases). These vanguard enzymes are encoded in gene clusters that code for diverse SQ catabolic pathways. To identify, visualize and isolate glycoside hydrolase CAZY‐family 31 (GH31) SQases in complex biological environments, we introduce SQ cyclophellitol‐aziridine activity‐based probes (ABPs). These ABPs label the active site nucleophile of this enzyme family, consistent with specific recognition of the SQ cyclophellitol‐aziridine in the active site, as evidenced in the 3D structure of Bacillus megaterium SQase. A fluorescent Cy5‐probe enables visualization of SQases in crude cell lysates from bacteria harbouring different SQ breakdown pathways, whilst a biotin‐probe enables SQase capture and identification by proteomics. The Cy5‐probe facilitates monitoring of active SQase levels during different stages of bacterial growth which show great contrast to more traditional mRNA analysis obtained by RT‐qPCR. Given the importance of SQases in global sulfur cycling and in human microbiota, these SQase ABPs provide a new tool with which to study SQase occurrence, activity and stability. [ABSTRACT FROM AUTHOR]

Glycoside hydrolases (glycosidases) take part in myriad biological processes and are important therapeutic targets. Competitive and mechanism‐based inhibitors are useful tools to dissect their biological role and comprise a good starting point for drug discovery. The natural product, cyclophellitol, a mechanism‐based, covalent and irreversible retaining β‐glucosidase inhibitor has inspired the design of diverse α‐ and β‐glycosidase inhibitor and activity‐based probe scaffolds. Here, we sought to deepen our understanding of the structural and functional requirements of cyclophellitol‐type compounds for effective human α‐glucosidase inhibition. We synthesized a comprehensive set of α‐configured 1,2‐ and 1,5a‐cyclophellitol analogues bearing a variety of electrophilic traps. The inhibitory potency of these compounds was assessed towards both lysosomal and ER retaining α‐glucosidases. These studies revealed the 1,5a‐cyclophellitols to be the most potent retaining α‐glucosidase inhibitors, with the nature of the electrophile determining inhibitory mode of action (covalent or non‐covalent). DFT calculations support the ability of the 1,5a‐cyclophellitols, but not the 1,2‐congeners, to adopt conformations that mimic either the Michaelis complex or transition state of α‐glucosidases. [ABSTRACT FROM AUTHOR]

BackgroundEmphysematous pyelonephritis is a severe infection of the kidney associated with formation of gas in the renal parenchyma and/or collecting system. The purpose of this study was to evaluate outcomes of patients with emphysematous pyelonephritis in a contemporary cohort and to evaluate the impact of urolithiasis on disease severity.MethodsA search of all imaging reports at University of California San Francisco (UCSF) for the term "emphysematous pyelonephritis" was undertaken from 2003-2014. Patients were included if there was clinical evidence of infection, no recent urologic instrumentation, and computerized tomography (CT) demonstrating gas in the renal parenchyma or collecting system. Clinical and laboratory variables were obtained from medical records.ResultsA total of 14 cases were identified. The majority of patients (57%) had gas confined to the collecting system. Three patients (21%) had gas in the renal parenchyma and 3 patients (21%) had gas extending into perirenal tissues. A total of 8 patients (57%) had concomitant urolithiasis. Seven patients (50%) were managed with antibiotic therapy alone while 6 patients (43%) required percutaneous drainage. No patients required immediate nephrectomy. There were no deaths. Patients with urolithiasis had less severe emphysematous pyelonephritis than patients without urolithiasis (P

Individuals with Netherton syndrome (NTS) have increased serine protease activity, which strongly impacts the barrier function of the skin epidermis and leads to skin inflammation. Here, we investigated how serine protease activity in NTS correlates with changes in the stratum corneum (SC) ceramides, which are crucial components of the skin barrier. We examined two key enzymes involved in epidermal ceramide biosynthesis, β-glucocerebrosidase (GBA) and acid-sphingomyelinase (ASM). We compared in situ expression levels and activities of GBA and ASM between NTS patients and controls and correlated the expression and activities with i) SC ceramide profiles, ii) in situ serine protease activity, and iii) clinical presentation of patients. Using activity-based probe labeling, we visualized and localized active epidermal GBA, and a newly developed in situ zymography method enabled us to visualize and localize active ASM. Reduction in active GBA in NTS patients coincided with increased ASM activity, particularly in areas with increased serine protease activity. NTS patients with scaly erythroderma exhibited more pronounced anomalies in GBA and ASM activities than patients with ichthyosis linearis circumflexa. They also displayed a stronger increase in SC ceramides processed via ASM. We conclude that changes in the localization of active GBA and ASM correlate with i) altered SC ceramide composition in NTS patients, ii) local serine protease activity, and iii) the clinical manifestation of NTS.

Chemical tools to monitor drug-target engagement of endogenous enzymes are essential for preclinical target validation. Here, the authors present a chemical genetics strategy to study target engagement of endogenous kinases, achieving specific labeling and inactivation of FES kinase to provide insights into FES’ role in neutrophil phagocytosis.

Background: The natural transition from neonatal deciduous teeth to adult permanent teeth is a physiological phenomenon. Miura et al. reported the isolation and characterization of stem cells from the pulp of human exfoliated deciduous teeth (SHED). The great majority of current stem cell therapies use human adult stem cells. Since adult stem cells are multi-potent. SHED have demonstrated to have the capability to differentiate into osteogenic and odontogenic cells, adipocytes, and neural cells. When transplanted in immunocompromised mice, SHED were able to form bone. In addition, SHED have been able to differentiate into functional odontoblast and angiogenic endothelial cells. Aims and Objective: There are two objectives in our study; First, we want to confirm that SHED cells differentiate into osteogenic and neurotic cells. Second, we shall also examine whether SHED stem cells can also differentiate into cardiomyocytes. Material and Method: SHED stem cells are subjected to cardiomyogenic, neurogenic, and osteogenic induction treatment. The treated cells are the subjected to real time PCR and mmuno-histochemical analysis. The presence of calcium deposits with Alizarin Red S staining for SHED cells treated with osteogenic media was used to confirm osteogenic differentiation. Results: Our study confirmed that SHED cells differentiate into osteogenic and neurotic cells. For the first time we showed that SHED cells can also differentiate into cardiomyocytes. Conclusion: Due to their potentials and its neural crest origin, SHED are an ideal stem cell source for tissue regeneration.

Activity-based protein profiling (ABPP) has so far scarcely been applied in Archaea in general and, especially, in extremophilic organisms. We herein isolated a novel Thermococcus strain designated sp. strain 2319x1E derived from the same enrichment culture as the recently reported Thermococcus sp. strain 2319x1. Both strains are able to grow with xylan as the sole carbon and energy source, and for Thermococcus sp. strain 2319x1E (optimal growth at 85°C, pH 6–7), the induction of xylanolytic activity in the presence of xylan was demonstrated. Since the solely sequence-based identification of xylanolytic enzymes is hardly possible, we established a complementary approach by conducting comparative full proteome analysis in combination with ABPP using α- or β-glycosidase selective probes and subsequent mass spectrometry (MS)-based analysis. This complementary proteomics approach in combination with recombinant protein expression and classical enzyme characterization enabled the identification of a novel bifunctional maltose-forming α-amylase and deacetylase (EGDIFPOO_00674) belonging to the GH57 family and a promiscuous β-glycosidase (EGIDFPOO_00532) with β-xylosidase activity. We thereby further substantiated the general applicability of ABPP in archaea and expanded the ABPP repertoire for the identification of glycoside hydrolases in hyperthermophiles.

Pseudomonas aeruginosa, a pathogenic Gram-negative bacterium for which currently antibiotic resistance is posing a significant problem and for which no vaccines are available, protects itself by the formation of a biofilm. The Pel polysaccharide, a cationic polymer composed of cis-linked galactosamine (GalN), N-acetyl galactosamine (GalNAc), glucosamine (GlcN) and N-acetyl glucosamine (GlcNAc) monosaccharides, is an important constituent of the biofilm. Well-defined Pel oligosaccharides will be valuable tools to probe the biosynthesis machinery of this polysaccharide and may serve as diagnostic tools or be used as components of glycoconjugate vaccines. We here, report on the development of synthetic chemistry to access well-defined Pel-oligosaccharides. The chemistry hinges on the use of di-tert-butylsilylidene protected GalN and GlcN building blocks, which allow for completely cis-selective glycosylation reactions. We show the applicability of the chemistry by the assembly of a matrix of 3 × 6 Pel heptasaccharides, which has been generated from a single set of suitably protected Pel heptasaccharides, in which a single glucosamine residue is incorporated and positioned at different places along the Pel oligo-galactosamine chain.

ObjectiveTo describe epidemiologic features of sports-related genitourinary (GU) injuries and determine patient cohorts and particular sporting activities associated with increased GU injury risk.Materials and methodsThe National Electronic Injury Surveillance System, a data set validated to provide a probability sample of injury-related US emergency department (ED) presentations, was analyzed to characterize GU injuries between 2002 and 2010. A total of 13,851 observations were analyzed to derive national estimates.ResultsBetween 2002 and 2010, an estimated 137,525 individuals (95% confidence interval, 104,490-170,620) presented to US EDs with GU injuries sustained during sporting activities. Nearly three-quarters of injuries occurred in the pediatric population. The most common product involved was a bicycle, representing approximately one-third of injuries in both adult and pediatric populations. Injuries related to team sports such as football, baseball or softball, basketball, and soccer were also common, particularly among boys where they represented a combined third of all injuries. Eighty-nine percent of all patients were evaluated and treated in the ED without inpatient admission. The large majority of injuries involved the external genitalia (60%), and significant injuries of paired GU organs (kidneys and testicles) requiring inpatient admission were rare (8.5%).ConclusionSports-related GU injuries are most commonly sustained during the use of a bicycle. However, there are other associated activities with identifiable high-risk cohorts, products, and situations. Consumers, practitioners, and injury-prevention experts can use our epidemiologic data to prioritize and develop strategies aimed at the prevention and limitation of such injuries, particularly when counseling at-risk cohorts, such as those with solitary kidneys or testicles.

Background. The increased usage of marginal grafts has triggered interest in perfused kidney preservation to minimize graft injury. We used a donation after circulatory death (DCD) porcine kidney autotransplantation model to compare 3 of the most frequently used ex vivo kidney perfusion techniques: nonoxygenated hypothermic machine perfusion (non-oxHMP), oxygenated hypothermic machine perfusion (oxHMP), and normothermic ex vivo kidney perfusion (NEVKP). Methods. Following 30 min of warm ischemia, grafts were retrieved and preserved with either 16 h of non-oxHMP, oxHMP, or NEVKP (n = 5 per group). After contralateral nephrectomy, grafts were autotransplanted and animals were followed for 8 d. Kidney function and injury markers were compared between groups. Results. NEVKP demonstrated a significant reduction in preservation injury compared with either cold preservation method. Grafts preserved by NEVKP showed superior function with lower peak serum creatinine (NEVKP versus non-oxHMP versus oxHMP: 3.66 ± 1.33 mg/dL, 8.82 ± 3.17 mg/dL, and 9.02 ± 5.5 mg/dL) and more rapid recovery. The NEVKP group demonstrated significantly increased creatinine clearance on postoperative day 3 compared with the cold perfused groups. Tubular injury scores on postoperative day 8 were similar in all groups. Conclusions. Addition of oxygen during HMP did not reduce preservation injury of DCD kidney grafts. Grafts preserved with prolonged NEVKP demonstrated superior initial graft function compared with grafts preserved with non-oxHMP or oxHMP in a model of pig DCD kidney transplantation.

The Energy Performance of Buildings Directive (EPBD) enhanced the sustainable improvement of dwellings in the European Union. Member states formulated measurable goals to improve the housing stock, and monitoring systems were developed to give insights into the improvements. In the Netherlands, non-profit housing associations agreed to improve the quality of their housing stock to an average Dutch energy label B (energy index (EI NV) = 1.40) by 2020. Research assessing this progress over time is presented using an annual monitoring system based on 2.0 million energy performance calculations of 264 Dutch non-profit housing associations between 2017 and 2020. The assessment includes: a detailed description of the development of the state of the stock over time; the effect of changes to the stock (construction and demolition) and changes within the stock (different types of retrofit measures); and the different characteristics of non-profit housing associations. Insights from this research show which specific retrofit and other measures are adopted and have substantial impact over time. This provides a useful frame of reference for building stock analysis and accelerating the improvement of the building stock. It also creates a baseline of information for the future sustainable development of this particular stock. 'Practice relevance' This research reveals which energy saving measures are most and least employed over time in Dutch non-profit housing associations sector. Large urban housing associations own a large share of the Dutch non-profit housing stock, and their dwellings have on average a lower energy rating. However, the improvement of their dwellings between 2017 and 2020 is higher than for smaller housing associations, which already have on average a higher energy rating. While the construction and demolition of dwellings contribute to 15.6% of the annual improvement, most of the improvement of the energy performance depends on retrofitting the existing stock. The trends are found to rely most on traditional measures ('e.g'. the installation of high-efficiency gas boilers and improved insulation). However, the rate of adding photovoltaic (PV) solar systems has increased rapidly in recent years, while futureproof systems ('e.g'. heat pumps and district heating) only have a steady adoption rate in this sector.

Glioblastoma remains the most lethal form of brain cancer, where hybrid nanomaterials biofunctionalized with polysaccharide peptides offer disruptive strategies relying on passive/active targeting and multimodal therapy for killing cancer cells. Thus, in this research, we report for the first time the rational design and synthesis of novel hybrid colloidal nanostructures composed of gold nanoparticles stabilized by trisodium citrate (AuNP@TSC) as the oxidase-like nanozyme, coupled with cobalt-doped superparamagnetic iron oxide nanoparticles stabilized by carboxymethylcellulose ligands (Co-MION@CMC) as the peroxidase-like nanozyme. They formed inorganic–inorganic dual-nanozyme systems functionalized by a carboxymethylcellulose biopolymer organic shell, which can trigger a biocatalytic cascade reaction in the cancer tumor microenvironment for the combination of magnetothermal–chemodynamic therapy. These nanoassemblies were produced through a green aqueous process under mild conditions and chemically biofunctionalized with integrin-targeting peptide (iRDG), creating bioengineered nanocarriers. The results demonstrated that the oxidase-like nanozyme (AuNP) was produced with a crystalline face-centered cubic nanostructure, spherical morphology (diameter = 16 ± 3 nm), zeta potential (ZP) of −50 ± 5 mV, and hydrodynamic diameter (DH) of 15 ± 1 nm. The peroxide-like nanostructure (POD, Co-MION@CMC) contained an inorganic crystalline core of magnetite and had a uniform spherical shape (2R = 7 ± 1 nm) which, summed to the contribution of the CMC shell, rendered a hydrodynamic diameter of 45 ± 4 nm and a negative surface charge (ZP = −41 ± 5 mV). Upon coupling both nanozymes, water-dispersible colloidal supramolecular vesicle-like organic–inorganic nanostructures were produced (AuNP//Co-MION@CMC, ZP = −45 ± 4 mV and DH = 28 ± 3 nm). They confirmed dual-nanozyme cascade biocatalytic activity targeted by polymer–peptide conjugates (AuNP//Co-MION@CMC_iRGD, ZP = −29 ± 3 mV and DH = 60 ± 4 nm) to kill brain cancer cells (i.e., bioenergy “starvation” by glucose deprivation and oxidative stress through reactive oxygen species generation), which was boosted by the magneto-hyperthermotherapy effect when submitted to the alternating magnetic field (i.e., induced local thermal stress by “nanoheaters”). This groundwork offers a wide avenue of opportunities to develop innovative theranostic nanoplatforms with multiple integrated functionalities for fighting cancer and reducing the harsh side effects of conventional chemotherapy.

Among children, the incidence of bicycle-related genitourinary (GU) injuries was 448 per 100 000 (95% CI 383 to 514) and, among adults, was 53 per 100 000 (95% CI 36 to 71). Although children sustained more injuries, adults were more likely to being admitted to the hospital for the GU injury (OR 1.95, 95% CI 1.13 to 3.37). Children sustain nearly 10 times more GU injuries due to bicycles than adults, but adults have higher odds of sustaining injuries requiring admission.

PurposeA variety of clinical and imaging findings are used by clinicians to determine utility of renal angioembolization (AE) in managing renal trauma. Our purpose was to investigate specific criteria that clinicians who manage high-grade renal trauma (HGRT) utilize in decision-making for primary or delayed AE.MethodsA total of 413 urologists and interventional radiologists (IRs) who practice at level 1 or 2 trauma centers within the United States were provided an original survey via email on experience and opinions regarding the utility of AE for HGRT. We described overall practice patterns and assessed differences by clinician type, using the Fisher's exact test.ResultsA total of 79 (20 %) clinicians completed the survey. All clinicians had AE capability for HGRT management. A higher proportion of IRs reported using AE for grade I-II (33 vs. 3 %, p = 0.002), grade III (65 vs. 26 %, p = 0.001), and penetrating injuries (83 vs. 58 %, p = 0.02). A greater proportion of urologists reported using AE for grade V injuries (81 vs. 56 %, p = 0.03). Clinicians most commonly cited computed tomography evidence of active arterial bleeding (97 %), or arteriovenous fistula/pseudoaneurysm (94 %) as indications for primary AE, and 62 % identified concurrent visceral injury as factor that would necessitate surgical intervention.ConclusionIn a survey of clinicians, we report that IRs and urologists utilize AE differently when managing HGRT, as a higher proportion of IRs use AE to manage lower grade as well as penetrating injuries. Validation studies are needed to establish algorithms to identify patients with HGRT who would benefit from selective renal AE.

μ-glucosidases [GBA1 (glucocerebrosidase) and GBA2] are ubiquitous essential enzymes. Lysosomal GBA1 and cytosol-facing GBA2 degrade glucosylceramide (GlcCer); GBA1 deficiency causes Gaucher disease, a lysosomal storage disorder characterized by lysosomal accumulation of GlcCer, which is partly converted to glucosylsphingosine (GlcSph). GBA1 and GBA2 also may transfer glucose from GlcCer to cholesterol, yielding glucosylated cholesterol (GlcChol). Here, we aimed to clarify the role of zebrafish Gba2 in glycosphingolipid metabolism during Gba1 deficiency in zebrafish (Danio rerio), which are able to survive total Gba1 deficiency. We developed Gba1 (gba1−/−), Gba2 (gba2−/−), and double (gba1−/−:gba2−/−) zebrafish knockouts using CRISPR/Cas9 and explored the effects of both genetic and pharmacological interventions on GlcCer metabolism in individual larvae. Activity-based probes and quantification of relevant glycolipid metabolites confirmed enzyme deficiency. GlcSph increased in gba1−/− larvae (0.09 pmol/fish) but did not increase more in gba1−/−:gba2−/− larvae. GlcCer was comparable in gba1−/− and WT larvae but increased in gba2−/− and gba1−/−:gba2−/− larvae. Independent of Gba1 status, GlcChol was low in all gba2−/− larvae (0.05 vs. 0.18 pmol/fish in WT). Pharmacologic inactivation of zebrafish Gba1 comparably increased GlcSph. Inhibition of GlcCer synthase (GCS) in Gba1-deficient larvae reduced GlcCer and GlcSph, and concomitant inhibition of GCS and Gba2 with iminosugars also reduced excessive GlcChol. Finally, overexpression of human GBA1 and injection of recombinant GBA1 both decreased GlcSph. We determined that zebrafish larvae offer an attractive model to study glucosidase actions in glycosphingolipid metabolism in vivo, and we identified distinguishing characteristics of zebrafish Gba2 deficiency.

Abstract Anthracenenitrile oxide undergoes 1,3‐dipolar cycloaddition reaction with propargyl bromide affording the expected isoxazole as single regioisomer, suitably synthetically elaborated and functionalized with a protected triple bond. The introduction of a bromine atom at the position C10 of the anthracene moiety allows for inserting a variety of aromatic and heterocyclic substituents through Suzuki coupling. A two‐way synthetic route can lead to simple isoxazole derivatives or, after N−O bond reductive cleavage and BF3 complexation, enamino ketone boron complexes. The photophysical properties of both the substituted isoxazoles and the corresponding boron complexes were investigated to show the potentialities for the employment as fluorescent tags in imaging techniques. The quite good quantum yield values confirm the suitability of these compounds in the cellular environment. Scope and limitations of the methodology are discussed.

Glycosyl hydrolases (GHs) are carbohydrate-active enzymes that hydrolyze a specific β-glycosidic bond in glycoconjugate substrates; β-glucosidases degrade glucosylceramide, a ubiquitous glycosphingolipid. GHs are grouped into structurally similar families that themselves can be grouped into clans. GH1, GH5, and GH30 glycosidases belong to clan A hydrolases with a catalytic (β/α)8 TIM barrel domain, whereas GH116 belongs to clan O with a catalytic (α/α)6 domain. In humans, GH abnormalities underlie metabolic diseases. The lysosomal enzyme glucocerebrosidase (family GH30), deficient in Gaucher disease and implicated in Parkinson disease etiology, and the cytosol-facing membrane-bound glucosylceramidase (family GH116) remove the terminal glucose from the ceramide lipid moiety. Here, we compare enzyme differences in fold, action, dynamics, and catalytic domain stabilization by binding site occupancy. We also explore other glycosidases with reported glycosylceramidase activity, including human cytosolic β-glucosidase, intestinal lactase-phlorizin hydrolase, and lysosomal galactosylceramidase. Last, we describe the successful translation of research to practice: recombinant glycosidases and glucosylceramide metabolism modulators are approved drug products (enzyme replacement therapies). Activity-based probes now facilitate the diagnosis of enzyme deficiency and screening for compounds that interact with the catalytic pocket of glycosidases. Future research may deepen the understanding of the functional variety of these enzymes and their therapeutic potential.

Abstract: Deficiency of glucocerebrosidase (GBA), a lysosomal β-glucosidase, causes Gaucher disease. The enzyme hydrolyzes β-glucosidic substrates and transglucosylates cholesterol to cholesterol-β-glucoside. Here we show that recombinant human GBA also cleaves β-xylosides and transxylosylates cholesterol. The xylosyl-cholesterol formed acts as an acceptor for the subsequent formation of di-xylosyl-cholesterol. Common mutant forms of GBA from patients with Gaucher disease with reduced β-glucosidase activity were similarly impaired in β-xylosidase, transglucosidase, and transxylosidase activities, except for a slightly reduced xylosidase/glucosidase activity ratio of N370S GBA and a slightly reduced transglucosylation/glucosidase activity ratio of D409H GBA. XylChol was found to be reduced in spleen from patients with Gaucher disease. The origin of newly identified XylChol in mouse and human tissues was investigated. Cultured human cells exposed to exogenous β-xylosides generated XylChol in a manner dependent on active lysosomal GBA but not the cytosol-facing β-glucosidase GBA2. We later sought an endogenous β-xyloside acting as donor in transxylosylation reactions, identifying xylosylated ceramide (XylCer) in cells and tissues that serve as donor in the formation of XylChol. UDP-glucosylceramide synthase (GCS) was unable to synthesize XylChol but could catalyze the formation of XylCer. Thus, food-derived β-D-xyloside and XylCer are potential donors for the GBA-mediated formation of XylChol in cells. The enzyme GCS produces XylCer at a low rate. Our findings point to further catalytic versatility of GBA and prompt a systematic exploration of the distribution and role of xylosylated lipids.