Description:

Size: 100ul

Catalog no.: bs-13312R-A350

Price: 380 EUR

Product details

Gene ID Number

2639

Target Antigen

GCDH

Modification Site

None

Tested applications

IF(IHC-P)

French translation

anticorps

Clonality

Polyclonal

Modification

Unmodified

Concentration

1ug per 1ul

Excitation emission

343nm/442nm

Conjugated with

ALEXA FLUOR® 350

Crossreactivity

Human, Mouse, Rat

Clone

Polyclonal antibody

Recommended dilutions

IF(IHC-P)(1:50-200)

Purification

Purified by Protein A.

Conjugation

Alexa Fluor,ALEXA FLUOR 350

Category

Conjugated Primary Antibodies

Also known as

Anti-GCDH PAb ALEXA FLUOR 350

Host Organism

Rabbit (Oryctolagus cuniculus)

Specificity

This is a highly specific antibody against GCDH.

Long name

GCDH Polyclonal Antibody, ALEXA FLUOR 350 Conjugated

Cross-reactive species details

Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.

Source

This antibody was obtained by immunization of the host with KLH conjugated synthetic peptide derived from human GCDH

Storage conditions

Store 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.

Synonyms

ACAD5; EC 1.3.99.7; GCD; Gcdh; GCDH_HUMAN; Glutaryl CoA dehydrogenase, mitochondrial; Glutaryl Coenzyme A dehydrogenase; Glutaryl-CoA dehydrogenase; mitochondrial; MS781.

Properties

For facs or microscopy Alexa 1 conjugate.Alexa Fluor 350 conjugates can be used in multi-color flow cytometry with FACS's equipped with a second red laser or red diode.If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies. ALEXA FLUOR they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.

Background of the antigen

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.