Anti-HMGCS2 Antibody (CAB19232)

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SKU:
CAB19232
Product type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Reactivity:
Mouse
Reactivity:
Rat
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
HMGCS2
Synonyms:
Hydroxymethylglutaryl-CoA synthase
Synonyms:
mitochondrial
Synonyms:
3-hydroxy-3-methylglutaryl coenzyme A synthase
Synonyms:
3-hydroxy-3-methylglutaryl-CoA synthase 2
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Description

抗体名:HMGCS2 Rabbit mAb
抗体コード:CAB19232
サイズ:20uL, 50uL, 100uL
同義語:HMGCS2, Hydroxymethylglutaryl-CoA synthase, mitochondrial, 3-hydroxy-3-methylglutaryl coenzyme A synthase, 3-hydroxy-3-methylglutaryl-CoA synthase 2
申し込み:WB, IHC
反応性:Human, Mouse, Rat
宿主種:Rabbit
免疫原:Recombinant protein of human HMGCS2.
申し込み:WB, IHC
推奨される希釈:WB 1:500 - 1:2000 IHC 1:50 - 1:200
反応性:Human, Mouse, Rat
ポジティブサンプル:HepG2, Mouse liver, Rat liver
免疫原:Recombinant protein of human HMGCS2.
精製方法:Affinity purification
保管所: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:Email for sequence
遺伝子ID:3158
Uniprot:P54868
セルラーロケーション:Mitochondrion
計算された分子量:50kDa
観察された分子量:50KDa
UniProt Protein Function:HMGCS2: This enzyme condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA, which is the substrate for HMG-CoA reductase. Defects in HMGCS2 are the cause of HMG-CoA synthase deficiency (HMGCS deficiency); also known as deficiency of mitochondrial 3-hydroxy-3-methylglutaryl-CoA synthase 2. Affected individuals present with severe hypoketotic hypoglycemia, mild hepatomegaly, or fatty liver, and a nondiagnostic pattern of urinary organic acids with increase of medium and short chain dicarboxylic acids. Belongs to the HMG-CoA synthase family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Transferase; Mitochondrial; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.3.10; Carbohydrate Metabolism - butanoate; Secondary Metabolites Metabolism - terpenoid backbone biosynthesis; Lipid Metabolism - synthesis and degradation of ketone bodies

Chromosomal Location of Human Ortholog: 1p13-p12

Cellular Component: mitochondrial matrix; mitochondrion

Molecular Function:hydroxymethylglutaryl-CoA synthase activity

Biological Process: ketone body biosynthetic process

Disease: 3-hydroxy-3-methylglutaryl-coa Synthase 2 Deficiency

NCBI Summary:The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009]
UniProt Code:P54868
NCBI GenInfo Identifier:1708234
NCBI Gene ID:3158
NCBI Accession:P54868.1
UniProt Secondary Accession:P54868,Q5SZU2, Q6IBF4, B7Z8R3, D3Y5K6,
UniProt Related Accession:P54868
Molecular Weight:50,050 Da
NCBI Full Name:Hydroxymethylglutaryl-CoA synthase, mitochondrial
NCBI Synonym Full Names:3-hydroxy-3-methylglutaryl-CoA synthase 2
NCBI Official Symbol:HMGCS2  
NCBI Protein Information:hydroxymethylglutaryl-CoA synthase, mitochondrial
UniProt Protein Name:Hydroxymethylglutaryl-CoA synthase, mitochondrial
UniProt Synonym Protein Names:3-hydroxy-3-methylglutaryl coenzyme A synthase
UniProt Gene Name:HMGCS2  
UniProt Entry Name:HMCS2_HUMAN
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