NDUFV1 Antibody (PACO44840)
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주문- SKU:
- PACO44840
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | NDUFV1 Antibody (PACO44840) |
Antibody SKU: | PACO44840 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, WB, IHC |
Recommended Dilutions: | ELISA:1:2000-1:10000, WB:1:1000-1:5000, IHC:1:20-1:200 |
Species Reactivity: | Human |
Immunogen: | Recombinant Human NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial protein (1-250AA) |
Form: | Liquid |
Storage Buffer: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Purification Method: | Antigen Affinity Purified |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | Western blot. All lanes: NDUFV1 antibody at 3.46µg/ml + HepG2 whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 51, 50 kDa. Observed band size: 51 kDa.. |
![]() | Immunohistochemistry of paraffin-embedded human kidney tissue using PACO44840 at dilution of 1:100. |
![]() | Immunohistochemistry of paraffin-embedded human brain tissue using PACO44840 at dilution of 1:100. |
Background: | Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 (By similarity). |
Synonyms: | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial (EC 1.6.5.3) (EC 1.6.99.3) (Complex I-51kD) (CI-51kD) (NADH dehydrogenase flavoprotein 1) (NADH-ubiquinone oxidoreductase 51 kDa subunit), NDUFV1, UQOR1 |
UniProt Protein Function: | NDUFV1: Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for 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 NDUFV1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions. Defects in NDUFV1 are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease. Belongs to the complex I 51 kDa subunit family. 2 isoforms of the human protein are produced by alternative splicing. |
UniProt Protein Details: | Protein type:EC 1.6.99.3; EC 1.6.5.3; Energy Metabolism - oxidative phosphorylation; Mitochondrial; Oxidoreductase Chromosomal Location of Human Ortholog: 11q13 Cellular Component: mitochondrial inner membrane; mitochondrial respiratory chain complex I Molecular Function:NADH dehydrogenase (ubiquinone) activity; FMN binding; metal ion binding; 4 iron, 4 sulfur cluster binding; NAD binding Biological Process: cellular metabolic process; mitochondrial electron transport, NADH to ubiquinone Disease: Mitochondrial Complex I Deficiency |
NCBI Summary: | The mitochondrial respiratory chain provides energy to cells via oxidative phosphorylation and consists of four membrane-bound electron-transporting protein complexes (I-IV) and an ATP synthase (complex V). This gene encodes a 51 kDa subunit of the NADH:ubiquinone oxidoreductase complex I; a large complex with at least 45 nuclear and mitochondrial encoded subunits that liberates electrons from NADH and channels them to ubiquinone. This subunit carries the NADH-binding site as well as flavin mononucleotide (FMN)- and Fe-S-biding sites. Defects in complex I are a common cause of mitochondrial dysfunction; a syndrome that occurs in approximately 1 in 10,000 live births. Mitochondrial complex I deficiency is linked to myopathies, encephalomyopathies, and neurodegenerative disorders such as Parkinson's disease and Leigh syndrome. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Oct 2009] |
UniProt Code: | P49821 |
NCBI GenInfo Identifier: | 20455501 |
NCBI Gene ID: | 4723 |
NCBI Accession: | P49821.4 |
UniProt Related Accession: | P49821 |
Molecular Weight: | |
NCBI Full Name: | NADH dehydrogenase |
NCBI Synonym Full Names: | NADH:ubiquinone oxidoreductase core subunit V1 |
NCBI Official Symbol: | NDUFV1 |
NCBI Official Synonym Symbols: | UQOR1; CI-51K; CI51KD; MC1DN4 |
NCBI Protein Information: | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial |
UniProt Protein Name: | NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial |
UniProt Synonym Protein Names: | Complex I-51kD; CI-51kD; NADH dehydrogenase flavoprotein 1; NADH-ubiquinone oxidoreductase 51 kDa subunit |
UniProt Gene Name: | NDUFV1 |
UniProt Entry Name: | NDUV1_HUMAN |