- Catalog numberbs-13312R-A647
- Product nameGCDH Antibody, ALEXA FLUOR 647
- Size100ul
- PriceAsk For Price
- Short nameAnti-GCDH
- Long nameGCDH Polyclonal Antibody, ALEXA FLUOR 647 Conjugated
- Also known asAnti-GCDH PAb ALEXA FLUOR 647
- CategoryConjugated Primary Antibodies
- Conjugated withALEXA FLUOR® 647
- Host OrganismRabbit (Oryctolagus cuniculus)
- Target AntigenGCDH
- SpecificityThis is a highly specific antibody against GCDH.
- ModificationUnmodified
- Modification SiteNone
- ClonalityPolyclonal
- ClonePolyclonal antibody
- IsotypeImmunoglobulin G (IgG)
- Concentration1ug per 1ul
- SourceThis antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GCDH
- Gene ID Number2639
- Tested applicationsIF(IHC-P)
- Recommended dilutionsIF(IHC-P)(1:50-200)
- CrossreactivityHuman, Mouse, Rat
- Cross reactive species detailsDue to limited amount of testing and knowledge, not every possible cross-reactivity is known.
- Background of the antigenGCDH 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.
- PurificationPurified by Protein A.
- Storage conditionsStore this antibody in aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Keep refrigerated at 2 to 8 degrees Celcius for up to one year.
- Excitation emission650nm/665nm
- SynonymsACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781.
- Gene targetGCDH
- Identity:HGNC:4189
- Gene:GCDH
- Long gene name:glutaryl-CoA dehydrogenase
- Synonyms gene name:glutaryl-Coenzyme A dehydrogenase
- Synonyms:ACAD5GCD
- Discovery year:1992-12-17
- Entrez gene record:2639
- Pubmed identification:1438360, 8088809
- Classification:Acyl-CoA dehydrogenase family
- VEGA ID:OTTHUMG00000180561
- Gene symbolGCDH
- isotype filter
- Immunoglobulin G IgG
- LabelALEXA FLUOR 647
- label filter
- ALEXA FLUOR 647
- technique filter
- Antibody
- TechniqueAntibody, antibodies against human proteins, antibodies for
- Alternative techniqueantibodies