GCDH antibody (AA 201-300)
Quick Overview for GCDH antibody (AA 201-300) (ABIN1713809)
Target
See all GCDH AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- AA 201-300
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Predicted Reactivity
- Human,Mouse,Rat
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Purification
- Purified by Protein A.
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Immunogen
- KLH conjugated synthetic peptide derived from human GCDH
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Isotype
- IgG
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Application Notes
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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
ICC 1:100-500 -
Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 1 μg/μL
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Buffer
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
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Preservative
- ProClin
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Precaution of Use
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
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Storage
- 4 °C,-20 °C
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Storage Comment
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
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Expiry Date
- 12 months
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- GCDH (Glutaryl-CoA Dehydrogenase (GCDH))
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Alternative Name
- GCDH
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Background
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Synonyms: ACAD5, EC 1.3.99.7, GCD, Gcdh, GCDH_HUMAN, Glutaryl CoA dehydrogenase, mitochondrial, Glutaryl Coenzyme A dehydrogenase, Glutaryl-CoA dehydrogenase, mitochondrial, MS781.
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.
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Gene ID
- 2639
Target
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