GCSH Polyclonal Antibody, AbBy Fluor™ 647 Conjugated
Product Specifications
Background
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.
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.
Gene ID
2653
Cellular Locus
Cytoplasm
Host
Rabbit
Immunogen
101-173/173
Target
GCSH
Clonality
Polyclonal
Isotype
IgG
Conjugation
AbBy Fluor™ 647
Source
KLH conjugated synthetic peptide derived from human GCSH
Applications
WB, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
650nm/665nm
Concentration
1µg/µl
Dilution
WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
2653
Predicted Cross Reactivity
Human, Mouse, Rat, Horse
Curated Selection
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