Description:

Size: 100ul

Catalog no.: bs-13323R-A594

Price: 380 EUR

Product details

Gene ID Number

2653

Target Antigen

GCSH

Modification Site

None

Tested applications

IF(IHC-P)

French translation

anticorps

Modification

Unmodified

Clonality

Polyclonal

Excitation emission

590nm/617nm

Concentration

1ug per 1ul

Crossreactivity

Human, Mouse, Rat

Conjugated with

ALEXA FLUOR® 594

Conjugated

Alexa conjugate 1

Recommended dilutions

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

Clone

Polyclonal antibody

Purification

Purified by Protein A.

Category

Conjugated Primary Antibodies

Also known as

Anti-GCSH PAb ALEXA FLUOR 594

Conjugation

Alexa Fluor,ALEXA FLUOR® 594

Host Organism

Rabbit (Oryctolagus cuniculus)

Specificity

This is a highly specific antibody against GCSH.

Long name

GCSH Polyclonal Antibody, ALEXA FLUOR 594 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 GCSH

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.

Properties

For facs or microscopy Alexa 1 conjugate.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.

Synonyms

GCE; GCSH; GCSH_HUMAN; Glycine cleavage system H protein; Glycine cleavage system H protein mitochondrial; Glycine cleavage system protein H aminomethyl carrier; Glycine cleavage system protein H; Lipoic acid containing protein; mitochondrial; Mitochondrial glycine cleavage system H protein; NKH.

Background of the antigen

GCSH is a 173 amino acid mitochondrial protein that contains one lipoyl-binding domain and belongs to the gcvH family. Defects in the gene encoding GCSH are the cause of glycine encephalopathy (GCE), an autosomal recessive disease that is also referred to as non-ketotic hyperglycinemia (NKH). Characterized by severe neurological symptoms, patients with GCE have a large amount of glycine accumulated in their body fluids. The gene encoding GCSH maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome.