Anti-GCSH Antibody (CAB13695)
- SKU:
- CAB13695
- 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-GCSH Antibody |
抗体コード: | CAB13695 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2). |
申し込み: | WB IF |
推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | LO2, 293T, HeLa, Mouse kidney, Rat liver |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-173 of human GCSH (NP_004474.2). |
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: | 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 |
遺伝子ID: | 2653 |
Uniprot: | P23434 |
セルラーロケーション: | Mitochondrion |
計算された分子量: | 18kDa |
観察された分子量: | 19kDa |
同義語: | GCSH, GCE, NKH |
バックグラウンド: | 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 |
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