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Recombinant Human GCDH/GCD protein is a Human Full Length protein, in the 45 to 438 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.

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Images

SDS-PAGE - Recombinant Human GCDH/GCD protein (AB98118), expandable thumbnail

Key facts

Purity
>90% SDS-PAGE
Expression system
Escherichia coli
Tags
His tag N-Terminus
Applications
SDS-PAGE, MS
Biologically active
No

Amino acid sequence

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

Reactivity data

Application
SDS-PAGE
Reactivity
Reacts
Dilution info
-
Notes

-

Application
MS
Reactivity
Reacts
Dilution info
-
Notes

-

Target data

Function

Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.

Alternative names

Recommended products

Recombinant Human GCDH/GCD protein is a Human Full Length protein, in the 45 to 438 aa range, expressed in Escherichia coli, with >90% purity and suitable for SDS-PAGE, MS.

Key facts

Purity
>90% SDS-PAGE
Expression system
Escherichia coli
Applications
SDS-PAGE, MS
Accession
Q92947-1
Animal free
No
Species
Human
Concentration
Loading...
Storage buffer

pH: 8
Constituents: 20% Glycerol (glycerin, glycerine), 1.16% Sodium chloride, 0.316% Tris HCl, 0.077% (R*,R*)-1,4-Dimercaptobutan-2,3-diol

Sequence info

Amino acid sequence

M G S S H H H H H H S S G L V P R G S H M R P E F D W Q D P L V L E E Q L T T D E I L I R D T F R T Y C Q E R L M P R I L L A N R N E V F H R E I I S E M G E L G V L G P T I K G Y G C A G V S S V A Y G L L A R E L E R V D S G Y R S A M S V Q S S L V M H P I Y A Y G S E E Q R Q K Y L P Q L A K G E L L G C F G L T E P N S G S D P S S M E T R A H Y N S S N K S Y T L N G T K T W I T N S P M A D L F V V W A R C E D G C I R G F L L E K G M R G L S A P R I Q G K F S L R A S A T G M I I M D G V E V P E E N V L P G A S S L G G P F G C L N N A R Y G I A W G V L G A S E F C L H T A R Q Y A L D R M Q F G V P L A R N Q L I Q K K L A D M L T E I T L G L H A C L Q L G R L K D Q D K A A P E M V S L L K R N N C G K A L D I A R Q A R D M L G G N G I S D E Y H V I R H A M N L E A V N T Y E G T H D I H A L I L G R A I T G I Q A F T A S K
Accession
Q92947
Protein length
Full Length
Predicted molecular weight
45.8 kDa
Amino acids
45 to 438
Nature
Recombinant
Tags
His tag N-Terminus

Specifications

Form
Liquid
Additional notes

ab98118 is purified by using conventional chromatography techniques.

General info

Function

Catalyzes the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine, and L-tryptophan metabolism. It uses electron transfer flavoprotein as its electron acceptor. Isoform Short is inactive.

Sequence similarities

Belongs to the acyl-CoA dehydrogenase family.

Subcellular localisation
Mitochondrion matrix

Storage

Shipped at conditions
Blue Ice
Appropriate short-term storage conditions
-20°C
Appropriate long-term storage conditions
-20°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle

Notes

This product was previously labelled as GCDH

Supplementary info

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

GCDH also known as glutaryl-CoA dehydrogenase is an enzyme involved in the catabolic breakdown of specific amino acids. This protein harbors a mass of approximately 45 kDa and functions primarily within the mitochondria. GCDH expression occurs in various tissues including the liver kidney and brain. The enzyme plays a mechanical role by catalyzing the decarboxylation of glutaryl-CoA to crotonyl-CoA facilitating the removal of carbon dioxide in this biochemical process.

Biological function summary

This enzyme acts as an important component in the lysine hydroxylysine and tryptophan degradation pathways. GCDH does not form part of a larger enzyme complex but performs its function independently ensuring the proper degradation and metabolism of amino acids. Its activity aids in maintaining metabolic homeostasis preventing the accumulation of harmful intermediates within cells. GCDH may interact with cofactors like flavin adenine dinucleotide (FAD) to carry out its enzymatic activity.

Pathways

GCDH plays a significant role in the lysine and tryptophan metabolism pathways. Within these pathways GCDH helps convert glutaryl-CoA a molecule derived from amino acid catabolism into intermediates ultimately leading to energy production. The enzyme's functionality connects with other mitochondrial enzymes like isovaleryl-CoA dehydrogenase (IVD) which also participates in amino acid breakdown. Proper interaction and coordination within these pathways are essential for maintaining metabolic balance in the cell.

Associated diseases and disorders

GCDH is closely linked to glutaric aciduria type 1 (GA1) an inborn error of metabolism. This disorder arises from mutations in the GCDH gene leading to insufficient enzyme activity and subsequent buildup of glutaric acid. This accumulation can cause severe neurological damage over time if untreated. GA1 can show genetic connections with other metabolic disorders involving enzymes like medium-chain acyl-CoA dehydrogenase (MCAD). Understanding these interactions helps in diagnosing and managing such metabolic disorders.

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1 product image

  • SDS-PAGE - Recombinant Human GCDH/GCD protein (ab98118), expandable thumbnail

    SDS-PAGE - Recombinant Human GCDH/GCD protein (ab98118)

    SDS-PAGE analysis of ab98118 (3µg) under reducing condition and visualized by coomassie blue stain.

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Product protocols

For this product, it's our understanding that no specific protocols are required. You can:

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