Anti-GATM Antibody (CAB6598)
- SKU:
- CAB6598
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | Anti-GATM Antibody |
抗体コード: | CAB6598 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IHC IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 124-423 of human GATM (NP_001473.1). |
申し込み: | WB IHC IF |
推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | U-251MG, Mouse testis, Rat testis |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 124-423 of human GATM (NP_001473.1). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | 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 |
遺伝子ID: | 2628 |
Uniprot: | P50440 |
セルラーロケーション: | Cytoplasm, Intermembrane side, Mitochondrion inner membrane, Peripheral membrane protein |
計算された分子量: | 44kDa/48kDa |
観察された分子量: | 48KDa |
同義語: | GATM, AGAT, AT, CCDS3 |
バックグラウンド: | 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 |
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