Antibody Name:	Anti-GCSH Antibody
Antibody SKU:	CAB13695
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	LO2, 293T, HeLa, Mouse kidney, Rat liver

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	MALR VVRS VRAL LCTL RAVP SPAA PCPP RPWQ LGVG AVRT LRTG PALL SVRK FTEK HEWV TTEN GIGT VGIS NFAQ EALG DVVY CSLP EVGT KLNK QDEF GALE SVKA ASEL YSPL SGEV TEIN EALA ENPG LVNK SCYE DGWL IKMT LSNP SELD ELMS EEAY EKYI KSIE E
Gene ID:	2653
Uniprot:	P23434
Cellular Location:	Mitochondrion
Calculated MW:	18kDa
Observed MW:	19kDa

Synonyms:

GCSH, GCE, NKH

Background:

Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding, have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.

UniProt Protein Function:	GCSH: The glycine cleavage system catalyzes the degradation of glycine. The H protein shuttles the methylamine group of glycine from the P protein to the T protein. Defects in GCSH are a cause of non-ketotic hyperglycinemia (NKH); also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms. Belongs to the GcvH family.
UniProt Protein Details:	Chromosomal Location of Human Ortholog: 16q23.2 Cellular Component: mitochondrion; glycine cleavage complex Molecular Function:enzyme binding; aminomethyltransferase activity Biological Process: methylation; glycine decarboxylation via glycine cleavage system; glycine catabolic process Disease: Glycine Encephalopathy
NCBI Summary:	Degradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). The protein encoded by this gene is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in this gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for this gene. Also, several transcribed and non-transcribed pseudogenes of this gene exist throughout the genome.[provided by RefSeq, Jan 2010]
UniProt Code:	P23434
NCBI GenInfo Identifier:	311033385
NCBI Gene ID:	2653
NCBI Accession:	P23434.2
UniProt Related Accession:	P23434
Molecular Weight:
NCBI Full Name:	Glycine cleavage system H protein, mitochondrial
NCBI Synonym Full Names:	glycine cleavage system protein H
NCBI Official Symbol:	GCSH
NCBI Official Synonym Symbols:	GCE; NKH
NCBI Protein Information:	glycine cleavage system H protein, mitochondrial
UniProt Protein Name:	Glycine cleavage system H protein, mitochondrial
UniProt Synonym Protein Names:	Lipoic acid-containing protein
Protein Family:	Glycine cleavage system H-like protein
UniProt Gene Name:	GCSH
UniProt Entry Name:	GCSH_HUMAN

	Western blot analysis of extracts of various cell lines, using GCSH antibody (CAB13695) at 1:3000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
	Immunofluorescence analysis of C6 cells using GCSH Rabbit pAb (CAB13695) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of L929 cells using GCSH Rabbit pAb (CAB13695) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of U-2 OS cells using GCSH Rabbit pAb (CAB13695) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Confocal immunofluorescence analysis of U2OS cells using GCSH antibody (CAB13695) at dilution of 1:100 (60x lens). Blue: DAPI for nuclear staining.