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AB235716

重组人GBA蛋白

Recombinant Human GBA protein

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Recombinant Human GBA protein is a Human Full Length protein, in the 40 to 536 aa range, expressed in Escherichia coli, suitable for SDS-PAGE.

查看别名

GBA, GC, GLUC, GBA1, Lysosomal acid glucosylceramidase, Lysosomal acid GCase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, Beta-glucosylceramidase 1, Cholesterol glucosyltransferase, Cholesteryl-beta-glucosidase, D-glucosyl-N-acylsphingosine glucohydrolase, Glucosylceramidase beta 1, Imiglucerase, Lysosomal cholesterol glycosyltransferase, Lysosomal galactosylceramidase, Lysosomal glycosylceramidase, Beta-GC, SGTase

1 Images
SDS-PAGE - Recombinant Human GBA protein (AB235716)
  • SDS-PAGE

Supplier Data

SDS-PAGE - Recombinant Human GBA protein (AB235716)

(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel using ab235716.

关键信息

纯度

undefined SDS-PAGE

表达系统

Escherichia coli

标签

Tag free

应用

SDS-PAGE

applications

生物活性

No

访问

P04062

不含动物源

No

不含载体蛋白

No

种属

Human

存储溶液

pH: 7.2 - 7.4 Constituents: Tris buffer, 50% Glycerol (glycerin, glycerine)

storage-buffer

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反应性数据

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" } } }
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序列信息

[{"sequence":"ARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITSLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ","proteinLength":"Full Length","predictedMolecularWeight":"55.6 kDa","actualMolecularWeight":null,"aminoAcidEnd":536,"aminoAcidStart":40,"nature":"Recombinant","expressionSystem":"Escherichia coli","accessionNumber":"P04062","tags":[]}]
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性能和储存信息

运输条件
Blue Ice
推荐的短期储存条件
-20°C
推荐的长期储存条件
-20°C
储存信息
Avoid freeze / thaw cycle
False
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补充信息

This supplementary information is collated from multiple sources and compiled automatically.

GBA also known as glucosylceramidase is a lysosomal enzyme with a molecular mass of approximately 59 kDa. This enzyme breaks down glucosylceramide into glucose and ceramide. GBA is expressed predominantly in tissues with high metabolic activities such as the brain liver and spleen. Its function relies on its catalytic activity where substrates bind to its active site enabling the hydrolysis process necessary for maintaining cellular metabolism.
Biological function summary

GBA plays an important role in sphingolipid metabolism. It participates in the degradation of glycolipids within the lysosome contributing to lipid recycling. It acts independently rather than as a part of a major enzymatic complex. Through its role in degrading glucosylceramide GBA influences cellular homeostasis and bioenergetics ensuring balance in neural and systemic lipid levels.

Pathways

GBA’s enzymatic functions are integral to the glycosphingolipid metabolic pathway. It is involved in the downstream steps of the lysosomal degradation of glycosphingolipids. The pathway operates alongside other important proteins such as beta-glucosidase and CERT-related transfer proteins all of which contribute to membrane lipid organization and signal transduction processes.

GBA mutations are linked with Gaucher disease and Parkinson’s disease. In Gaucher disease deficient GBA activity leads to substrate accumulation resulting in hepatosplenomegaly and other systemic symptoms. Reduced GBA activity is also associated with increased alpha-synuclein aggregation in Parkinson’s disease implicating it in the pathogenesis of neurodegenerative disorders. The enzyme’s function in these diseases highlights its role in maintaining cellular equilibrium and signaling pathways.
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特殊说明

形式

Liquid

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常规信息

功能

Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed : 15916907, PubMed : 24211208, PubMed : 32144204, PubMed : 39395789, PubMed : 9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed : 27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed : 19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 32144204). GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine) (PubMed : 24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) (PubMed : 26724485). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 39395789). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed : 32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside (PubMed : 33361282). Can also metabolize plant glycosyl phytosterols such as glucosylstigmasterol (PubMed : 39395789).

序列相似性

Belongs to the glycosyl hydrolase 30 family.

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产品实验方案

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靶点信息

Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose (PubMed : 15916907, PubMed : 24211208, PubMed : 32144204, PubMed : 39395789, PubMed : 9201993). Plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed : 27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed : 19279011). Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 32144204). GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine) (PubMed : 24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl 3-beta-D-glucoside to ceramide (Probable) (PubMed : 26724485). Can also hydrolyze cholesteryl 3-beta-D-glucoside producing glucose and cholesterol (PubMed : 24211208, PubMed : 26724485, PubMed : 39395789). Catalyzes the hydrolysis of galactosylceramides/GalCers (such as beta-D-galactosyl-(1<->1')-N-acylsphing-4-enine), as well as the transfer of galactose between GalCers and cholesterol in vitro, but with lower activity than with GlcCers (PubMed : 32144204). Contrary to GlcCer and GalCer, xylosylceramide/XylCer (such as beta-D-xyosyl-(1<->1')-N-acylsphing-4-enine) is not a good substrate for hydrolysis, however it is a good xylose donor for transxylosylation activity to form cholesteryl 3-beta-D-xyloside (PubMed : 33361282). Can also metabolize plant glycosyl phytosterols such as glucosylstigmasterol (PubMed : 39395789).
See full target information GBA1
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搭配使用产品

Reagents, Controls & Accessories

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Primary Antibodies

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Secondary Antibodies

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