UQCRQ Antibody (PACO13106)
- SKU:
- PACO13106
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | UQCRQ Antibody (PACO13106) |
抗体コード: | PACO13106 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, WB, IHC |
推奨される希釈: | |
反応性: | Human, Mouse, Rat |
免疫原: | Human UQCRQ |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
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 |
シノニム: | ubiquinol-cytochrome c reductase, complex III subunit VII, 9.5kDa;UQCRQ;QCR8;QP-C;QPC ; |
UniProt Protein Function: | UQCRQ: This is a component of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is part of the mitochondrial respiratory chain. This subunit, together with cytochrome b, binds to ubiquinone. Defects in UQCRQ are a cause of mitochondrial complex III deficiency (MT-C3D). A disorder of the mitochondrial respiratory chain resulting in a highly variable phenotype depending on which tissues are affected. Clinical features include mitochondrial encephalopathy, psychomotor retardation, ataxia, severe failure to thrive, liver dysfunction, renal tubulopathy, muscle weakness and exercise intolerance. Belongs to the UQCRQ/QCR8 family. |
UniProt Protein Details: | Protein type:Mitochondrial; Oxidoreductase; Energy Metabolism - oxidative phosphorylation Chromosomal Location of Human Ortholog: 5q31.1 Cellular Component: mitochondrial inner membrane; mitochondrion Biological Process: mitochondrial electron transport, ubiquinol to cytochrome c Disease: Mitochondrial Complex Iii Deficiency, Nuclear Type 4 |
NCBI Summary: | This gene encodes a ubiquinone-binding protein of low molecular mass. This protein is a small core-associated protein and a subunit of ubiquinol-cytochrome c reductase complex III, which is part of the mitochondrial respiratory chain. [provided by RefSeq, Jul 2008] |
UniProt Code: | O14949 |
NCBI GenInfo Identifier: | 20141846 |
NCBI Gene ID: | 27089 |
NCBI Accession: | O14949.4 |
UniProt Secondary Accession: | O14949,Q5FVE2, Q9BV88, Q9T2V7, |
UniProt Related Accession: | O14949 |
Molecular Weight: | 9,906 Da |
NCBI Full Name: | Cytochrome b-c1 complex subunit 8 |
NCBI Synonym Full Names: | ubiquinol-cytochrome c reductase complex III subunit VII |
NCBI Official Symbol: | UQCRQ |
NCBI Official Synonym Symbols: | QPC; QCR8; QP-C; UQCR7; MC3DN4 |
NCBI Protein Information: | cytochrome b-c1 complex subunit 8 |
UniProt Protein Name: | Cytochrome b-c1 complex subunit 8 |
UniProt Synonym Protein Names: | Complex III subunit 8; Complex III subunit VIII; Ubiquinol-cytochrome c reductase complex 9.5 kDa protein; Ubiquinol-cytochrome c reductase complex ubiquinone-binding protein QP-C |
UniProt Gene Name: | UQCRQ |
UniProt Entry Name: | QCR8_HUMAN |