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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 and suitable for SDS-PAGE.

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Images

SDS-PAGE - Recombinant Human GBA protein (AB235716), expandable thumbnail

Key facts

Purity

SDS-PAGE

Expression system

Escherichia coli

Tags

Tag free

Applications

SDS-PAGE

Biologically active

No

Amino acid sequence

A R P C I P K S F G Y S S V V C V C N A T Y C D S F D P P T F P A L G T F S R Y E S T R S G R R M E L S M G P I Q A N H T G T G L L L T L Q P E Q K F Q K V K G F G G A M T D A A A L N I L A L S P P A Q N L L L K S Y F S E E G I G Y N I I R V P M A S C D F S I R T Y T Y A D T P D D F Q L H N F S L P E E D T K L K I P L I H R A L Q L A Q R P V S L L A S P W T S P T W L K T N G A V N G K G S L K G Q P G D I Y H Q T W A R Y F V K F L D A Y A E H K L Q F W A V T A E N E P S A G L L S G Y P F Q C L G F T P E H Q R D F I A R D L G P T L A N S T H H N V R L L M L D D Q R L L L P H W A K V V L T D P E A A K Y V H G I A V H W Y L D F L A P A K A T L G E T H R L F P N T M L F A S E A C V G S K F W E Q S V R L G S W D R G M Q Y S H S I I T S L L Y H V V G W T D W N L A L N P E G G P N W V R N F V D S P I I V D I T K D T F Y K Q P M F Y H L G H F S K F I P E G S Q R V G L V A S Q K N D L D A V A L M H P D G S A V V V V L N R S S K D V P L T I K D P A V G F L E T I S P G Y S I H T Y L W R R Q

Reactivity data

Application

SDS-PAGE

Reactivity

Reacts

Dilution info

-

Notes

-

Associated Products

Select an associated product type

2 products for Alternative Product

Target data

Function

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: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). 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).

Alternative names

Recommended products

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

Key facts

Purity

SDS-PAGE

Expression system

Escherichia coli

Applications

SDS-PAGE

Accession
P04062-1
Animal free

No

Species

Human

Concentration
Loading...
Storage buffer

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

Sequence info

Amino acid sequence

A R P C I P K S F G Y S S V V C V C N A T Y C D S F D P P T F P A L G T F S R Y E S T R S G R R M E L S M G P I Q A N H T G T G L L L T L Q P E Q K F Q K V K G F G G A M T D A A A L N I L A L S P P A Q N L L L K S Y F S E E G I G Y N I I R V P M A S C D F S I R T Y T Y A D T P D D F Q L H N F S L P E E D T K L K I P L I H R A L Q L A Q R P V S L L A S P W T S P T W L K T N G A V N G K G S L K G Q P G D I Y H Q T W A R Y F V K F L D A Y A E H K L Q F W A V T A E N E P S A G L L S G Y P F Q C L G F T P E H Q R D F I A R D L G P T L A N S T H H N V R L L M L D D Q R L L L P H W A K V V L T D P E A A K Y V H G I A V H W Y L D F L A P A K A T L G E T H R L F P N T M L F A S E A C V G S K F W E Q S V R L G S W D R G M Q Y S H S I I T S L L Y H V V G W T D W N L A L N P E G G P N W V R N F V D S P I I V D I T K D T F Y K Q P M F Y H L G H F S K F I P E G S Q R V G L V A S Q K N D L D A V A L M H P D G S A V V V V L N R S S K D V P L T I K D P A V G F L E T I S P G Y S I H T Y L W R R Q

Accession

P04062

Protein length

Full Length

Predicted molecular weight

55.6 kDa

Amino acids

40 to 536

Nature

Recombinant

Specifications

Form

Liquid

General info

Function

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: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). 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).

Sequence similarities

Belongs to the glycosyl hydrolase 30 family.

Subcellular localisation

Lysosome membrane

Storage

Shipped at conditions

Blue Ice

Appropriate short-term storage conditions

-20°C

Appropriate long-term storage conditions

-20°C

Storage information

Avoid freeze / thaw cycle

Supplementary info

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

Activity summary

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.

Associated diseases and disorders

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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1 product image

  • SDS-PAGE - Recombinant Human GBA protein (ab235716), expandable thumbnail

    SDS-PAGE - Recombinant Human GBA protein (ab235716)

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

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