NDUFA12 Antibody (PACO17787)
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주문- SKU:
- PACO17787
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | NDUFA12 Antibody (PACO17787) |
Antibody SKU: | PACO17787 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:1000-1:10000, IHC:1:50-1:200 |
Species Reactivity: | Human, Mouse |
Immunogen: | Synthetic peptide of human NDUFA12 |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
The image on the left is immunohistochemistry of paraffin-embedded Human brain tissue using PACO17787(NDUFA12 Antibody) at dilution 1/50, on the right is treated with synthetic peptide. (Original magnification: x200). |
Background: | This gene encodes a protein which is part of mitochondrial complex 1, part of the oxidative phosphorylation system in mitochondria. Complex 1 transfers electrons to ubiquinone from NADH which establishes a proton gradient for the generation of ATP. Mutations in this gene are associated with Leigh syndrome due to mitochondrial complex 1 deficiency. Pseudogenes of this gene are located on chromosomes 5 and 13. Alternative splicing results in multiple transcript variants. |
Synonyms: | NADH dehydrogenase (ubiquinone) 1 α subcomplex, 12 |
UniProt Protein Function: | NDUFA12: Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. Defects in NDUFA12 are the cause of Leigh syndrome (LS). An early-onset progressive neurodegenerative disorder characterized by the presence of focal, bilateral lesions in one or more areas of the central nervous system including the brainstem, thalamus, basal ganglia, cerebellum and spinal cord. Clinical features depend on which areas of the central nervous system are involved and include subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, and dysphagia. Belongs to the complex I NDUFA12 subunit family. |
UniProt Protein Details: | Protein type:Oxidoreductase; Mitochondrial; EC 1.6.99.3; EC 1.6.5.3 Chromosomal Location of Human Ortholog: 12q22 Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I Biological Process: mitochondrial electron transport, NADH to ubiquinone; mitochondrial respiratory chain complex I assembly; response to oxidative stress Disease: Leigh Syndrome |
NCBI Summary: | This gene encodes a protein which is part of mitochondrial complex 1, part of the oxidative phosphorylation system in mitochondria. Complex 1 transfers electrons to ubiquinone from NADH which establishes a proton gradient for the generation of ATP. Mutations in this gene are associated with Leigh syndrome due to mitochondrial complex 1 deficiency. Pseudogenes of this gene are located on chromosomes 5 and 13. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Apr 2012] |
UniProt Code: | Q9UI09 |
NCBI GenInfo Identifier: | 12229870 |
NCBI Gene ID: | 55967 |
NCBI Accession: | Q9UI09.1 |
UniProt Secondary Accession: | Q9UI09,Q53XX0, Q9BRV6, F8VQS7, |
UniProt Related Accession: | Q9UI09 |
Molecular Weight: | 7,173 Da |
NCBI Full Name: | NADH dehydrogenase |
NCBI Synonym Full Names: | NADH:ubiquinone oxidoreductase subunit A12 |
NCBI Official Symbol: | NDUFA12 |
NCBI Official Synonym Symbols: | B17.2; DAP13 |
NCBI Protein Information: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 |
UniProt Protein Name: | NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 |
UniProt Synonym Protein Names: | 13 kDa differentiation-associated protein; Complex I-B17.2; CI-B17.2; CIB17.2; NADH-ubiquinone oxidoreductase subunit B17.2 |
Protein Family: | NADH dehydrogenase |
UniProt Gene Name: | NDUFA12 |
UniProt Entry Name: | NDUAC_HUMAN |