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GCDH Polyclonal Antibody

CAT: 0498-bs-13312RSize: 100 µLDry Ice: NoHazardous: No
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CAT#:0498-bs-13312RSize:100 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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Background
GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze 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. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.
Synonyms
ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781.
Gene ID
2639
Cellular Locus
Cytoplasm
Host
Rabbit
Immunogen
201-300/438
Target
GCDH
Clonality
Polyclonal
Isotype
IgG
Conjugation
Unconjugated
Source
KLH conjugated synthetic peptide derived from human GCDH
Applications
WB, ELISA, IHC-P, IHC-F, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
WB (1:300-5000), ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
Gene ID URL
2639
Predicted Cross Reactivity
Human, Mouse, Rat

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