Antibody Name:	Anti-GATM Antibody
Antibody SKU:	CAB6598
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 124-423 of human GATM (NP_001473.1).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	U-251MG, Mouse testis, Rat testis

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 124-423 of human GATM (NP_001473.1).
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:	VAEI EEMC NILK TEGV TVRR PDPI DWSL KYKT PDFE STGL YSAM PRDI LIVV GNEI IEAP MAWR SRFF EYRA YRSI IKDY FHRG AKWT TAPK PTMA DELY NQDY PIHS VEDR HKLA AQGK FVTT EFEP CFDA ADFI RAGR DIFA QRSQ VTNY LGIE WMRR HLAP DYRV HIIS FKDP NPMH IDAT FNII GPGI VLSN PDRP CHQI DLFK KAGW TIIT PPTP IIPD DHPL WMSS KWLS MNVL MLDE KRVM VDAN EVPI QKMF EKLG ITTI KVNI RNAN SLGG GFHC WTCD VRRR GTLQ SYLD
Gene ID:	2628
Uniprot:	P50440
Cellular Location:	Cytoplasm, Intermembrane side, Mitochondrion inner membrane, Peripheral membrane protein
Calculated MW:	44kDa/48kDa
Observed MW:	48KDa

Synonyms:

GATM, AGAT, AT, CCDS3

Background:

This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders.

UniProt Protein Function:	GATM: Catalyzes the biosynthesis of guanidinoacetate, the immediate precursor of creatine. Creatine plays a vital role in energy metabolism in muscle tissues. May play a role in embryonic and central nervous system development. May be involved in the response to heart failure by elevating local creatine synthesis. Defects in GATM are the cause of arginine:glycine amidinotransferase deficiency (AGAT deficiency). AGAT deficiency is an autosomal recessive disorder characterized by developmental delay/regression, mental retardation, severe disturbance of expressive and cognitive speech, and severe depletion of creatine/phosphocreatine in the brain. Belongs to the amidinotransferase family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Amino Acid Metabolism - arginine and proline; Amino Acid Metabolism - glycine, serine and threonine; Transferase; EC 2.1.4.1; Mitochondrial Chromosomal Location of Human Ortholog: 15q21.1 Cellular Component: mitochondrial inner membrane; mitochondrial intermembrane space Molecular Function:hydrolase activity, acting on carbon-nitrogen (but not peptide) bonds, in linear amidines; glycine amidinotransferase activity Biological Process: response to mercury ion; response to peptide hormone stimulus; creatine biosynthetic process; tissue regeneration; response to oxidative stress; creatine metabolic process; response to nutrient Disease: Cerebral Creatine Deficiency Syndrome 3
NCBI Summary:	This gene encodes a mitochondrial enzyme that belongs to the amidinotransferase family. This enzyme is involved in creatine biosynthesis, whereby it catalyzes the transfer of a guanido group from L-arginine to glycine, resulting in guanidinoacetic acid, the immediate precursor of creatine. Mutations in this gene cause arginine:glycine amidinotransferase deficiency, an inborn error of creatine synthesis characterized by mental retardation, language impairment, and behavioral disorders. [provided by RefSeq, Jul 2008]
UniProt Code:	P50440
NCBI GenInfo Identifier:	1730201
NCBI Gene ID:	2628
NCBI Accession:	P50440.1
UniProt Secondary Accession:	P50440,Q53EQ4, B4DH99, B4DPI3,
UniProt Related Accession:	P50440
Molecular Weight:	423
NCBI Full Name:	Glycine amidinotransferase, mitochondrial
NCBI Synonym Full Names:	glycine amidinotransferase (L-arginine:glycine amidinotransferase)
NCBI Official Symbol:	GATM
NCBI Official Synonym Symbols:	AT; AGAT; CCDS3
NCBI Protein Information:	glycine amidinotransferase, mitochondrial; transamidinase
UniProt Protein Name:	Glycine amidinotransferase, mitochondrial
UniProt Synonym Protein Names:	L-arginine:glycine amidinotransferase; Transamidinase
Protein Family:	Glycine amidinotransferase
UniProt Gene Name:	GATM
UniProt Entry Name:	GATM_HUMAN

	Western blot analysis of extracts of various cell lines, using GATM Rabbit pAb (CAB6598) 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: 1s.
	Immunohistochemistry of paraffin-embedded human liver using GATM Rabbit pAb (CAB6598) at dilution of 1:100 (40x lens).
	Immunofluorescence analysis of A549 cells using GATM antibody (CAB6598). Blue: DAPI for nuclear staining.