ATPAF2 Antibody, HRP conjugated (PACO25165)
- SKU:
- PACO25165
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | ATPAF2 Antibody, HRP conjugated (PACO25165) |
抗体コード: | PACO25165 |
サイズ: | 50ug |
宿主種: | Rabbit |
申し込み: | ELISA |
推奨される希釈: | |
反応性: | Human |
免疫原: | Recombinant Human ATP synthase mitochondrial F1 complex assembly factor 2 protein (41-289AA) |
憲法: | Liquid |
ストレージバッファ: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
精製方法: | >95%, Protein G purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | HRP |
シノニム: | ATP synthase mitochondrial F1 complex assembly factor 2 (ATP12 homolog), ATPAF2, ATP12 |
UniProt Protein Function: | ATPAF2: May play a role in the assembly of the F1 component of the mitochondrial ATP synthase (ATPase). Defects in ATPAF2 are a cause of mitochondrial complex V deficiency nuclear type 1 (MC5DN1). A mitochondrial disorder with heterogeneous clinical manifestations including dysmorphic features, psychomotor retardation, hypotonia, growth retardation, cardiomyopathy, enlarged liver, hypoplastic kidneys and elevated lactate levels in urine, plasma and cerebrospinal fluid. Belongs to the ATP12 family. |
UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 17p11.2 Cellular Component: cytosol; nuclear speck Molecular Function:protein binding Disease: Mitochondrial Complex V (atp Synthase) Deficiency, Nuclear Type 1 |
NCBI Summary: | This gene encodes an assembly factor for the F(1) component of the mitochondrial ATP synthase. This protein binds specifically to the F1 alpha subunit and is thought to prevent this subunit from forming nonproductive homooligomers during enzyme assembly. This gene is located within the Smith-Magenis syndrome region on chromosome 17. An alternatively spliced transcript variant has been described, but its biological validity has not been determined. [provided by RefSeq, Jul 2008] |
UniProt Code: | Q8N5M1 |
NCBI GenInfo Identifier: | 21735485 |
NCBI Gene ID: | 91647 |
NCBI Accession: | NP_663729.1 |
UniProt Secondary Accession: | Q8N5M1,Q6XYC7, A6NDE5, A8K2J2, |
UniProt Related Accession: | Q8N5M1 |
Molecular Weight: | 32.6kD |
NCBI Full Name: | ATP synthase mitochondrial F1 complex assembly factor 2 |
NCBI Synonym Full Names: | ATP synthase mitochondrial F1 complex assembly factor 2 |
NCBI Official Symbol: | ATPAF2 |
NCBI Official Synonym Symbols: | ATP12; ATP12p; LP3663; MC5DN1 |
NCBI Protein Information: | ATP synthase mitochondrial F1 complex assembly factor 2 |
UniProt Protein Name: | ATP synthase mitochondrial F1 complex assembly factor 2 |
UniProt Synonym Protein Names: | ATP12 homolog |
Protein Family: | ATP synthase |
UniProt Gene Name: | ATPAF2 |