Description
| 抗体名: | SCO2 Antibody (PACO44455) |
| 抗体コード: | PACO44455 |
| サイズ: | 50ul |
| 宿主種: | Rabbit |
| 申し込み: | ELISA, IHC |
| 推奨される希釈: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
| 反応性: | Human |
| 免疫原: | Recombinant Human Protein SCO2 homolog, mitochondrial protein (1-130AA) |
| 憲法: | Liquid |
| ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| 精製方法: | Antigen Affinity Purified |
| 抗体のクローン性: | Polyclonal |
| アイソタイプ: | IgG |
| Conjugate: | Non-conjugated |
 | Immunohistochemistry of paraffin-embedded human adrenal gland tissue using PACO44455 at dilution of 1:100. |
 | Immunohistochemistry of paraffin-embedded human gastric cancer using PACO44455 at dilution of 1:100. |
| バックグラウンド: | Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2). |
| シノニム: | Protein SCO2 homolog, mitochondrial, SCO2 |
| UniProt Protein Function: | SCO2: Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2). Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC). This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies. Belongs to the SCO1/2 family. |
| UniProt Protein Details: | Protein type:Mitochondrial Chromosomal Location of Human Ortholog: 22q13.33 Cellular Component: nucleoplasm; myofibril; mitochondrion; mitochondrial inner membrane Molecular Function:copper ion binding Biological Process: cellular copper ion homeostasis; eye development; in utero embryonic development; respiratory chain complex IV assembly; response to activity; muscle system process; copper ion transport Disease: Cardioencephalomyopathy, Fatal Infantile, Due To Cytochrome C Oxidase Deficiency 1; Myopia 6 |
| NCBI Summary: | Cytochrome c oxidase (COX) catalyzes the transfer of electrons from cytochrome c to molecular oxygen, which helps to maintain the proton gradient across the inner mitochondrial membrane that is necessary for aerobic ATP production. Human COX is a multimeric protein complex that requires several assembly factors; this gene encodes one of the COX assembly factors. The encoded protein is a metallochaperone that is involved in the biogenesis of cytochrome c oxidase subunit II. Mutations in this gene are associated with fatal infantile encephalocardiomyopathy and myopia 6. [provided by RefSeq, Oct 2014] |
| UniProt Code: | O43819 |
| NCBI GenInfo Identifier: | 8134662 |
| NCBI Gene ID: | 9997 |
| NCBI Accession: | O43819.3 |
| UniProt Secondary Accession: | O43819,Q3T1B5, Q9UK87, |
| UniProt Related Accession: | O43819 |
| Molecular Weight: | 29,810 Da |
| NCBI Full Name: | Protein SCO2 homolog, mitochondrial |
| NCBI Synonym Full Names: | SCO2 cytochrome c oxidase assembly protein |
| NCBI Official Symbol: | SCO2 |
| NCBI Official Synonym Symbols: | MYP6; SCO1L; CEMCOX1 |
| NCBI Protein Information: | protein SCO2 homolog, mitochondrial; SCO cytochrome oxidase deficient homolog 2 |
| UniProt Protein Name: | Protein SCO2 homolog, mitochondrial |
| Protein Family: | SCO2-like protein |
| UniProt Gene Name: | SCO2 |
| UniProt Entry Name: | SCO2_HUMAN |
