SCO1 Antibody, HRP conjugated (PACO47635)
- SKU:
- PACO47635
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Antibody type:
- Polyclonal
- Conjugation:
- HRP
Frequently bought together:
Description
抗体名: | SCO1 Antibody, HRP conjugated (PACO47635) |
抗体コード: | PACO47635 |
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 Protein SCO1 homolog, mitochondrial protein (29-176AA) |
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 |
バックグラウンド: | Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX. |
シノニム: | Protein SCO1 homolog, mitochondrial, SCO1, SCOD1 |
UniProt Protein Function: | SCO1: Thought to play a role in cellular copper homeostasis, mitochondrial redox signaling or insertion of copper into the active site of COX. Defects in SCO1 are a cause of mitochondrial complex IV deficiency (MT-C4D); also known as cytochrome c oxidase deficiency. A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations, ranging from isolated myopathy to severe multisystem disease affecting several tissues and organs. Features include hypertrophic cardiomyopathy, hepatomegaly and liver dysfunction, hypotonia, muscle weakness, excercise intolerance, developmental delay, delayed motor development and mental retardation. A subset of patients manifest Leigh syndrome. Belongs to the SCO1/2 family. |
UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 17p13.1 Cellular Component: mitochondrion; myofibril; mitochondrial inner membrane Molecular Function:copper ion binding Biological Process: generation of precursor metabolites and energy; cellular copper ion homeostasis; respiratory chain complex IV assembly; copper ion transport Disease: Mitochondrial Complex Iv Deficiency |
NCBI Summary: | Mammalian cytochrome c oxidase (COX) catalyzes the transfer of reducing equivalents from cytochrome c to molecular oxygen and pumps protons across the inner mitochondrial membrane. In yeast, 2 related COX assembly genes, SCO1 and SCO2 (synthesis of cytochrome c oxidase), enable subunits 1 and 2 to be incorporated into the holoprotein. This gene is the human homolog to the yeast SCO1 gene. [provided by RefSeq, Jul 2008] |
UniProt Code: | O75880 |
NCBI GenInfo Identifier: | 8134663 |
NCBI Gene ID: | 6341 |
NCBI Accession: | O75880.1 |
UniProt Secondary Accession: | O75880,B2RDM0, |
UniProt Related Accession: | O75880 |
Molecular Weight: | 33,814 Da |
NCBI Full Name: | Protein SCO1 homolog, mitochondrial |
NCBI Synonym Full Names: | SCO1 cytochrome c oxidase assembly protein |
NCBI Official Symbol: | SCO1 |
NCBI Official Synonym Symbols: | SCOD1 |
NCBI Protein Information: | protein SCO1 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 1 |
UniProt Protein Name: | Protein SCO1 homolog, mitochondrial |
Protein Family: | SCO1 protein |
UniProt Gene Name: | SCO1 |
UniProt Entry Name: | SCO1_HUMAN |