Anti-NDUFV1 Antibody (CAB8014)
- SKU:
- CAB8014
- Product type:
- Antibody
- Application:
- WB
- Application:
- IF
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
抗体名: | NDUFV1 Rabbit Polyclonal Antibody |
抗体コード: | CAB8014 |
抗体サイズ: | 20uL, 50uL, 100uL |
申し込み: | WB IF |
反応性: | Human, Mouse, Rat |
宿主種: | Rabbit |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2). |
申し込み: | WB IF |
推奨希釈: | WB 1:500 - 1:2000 IF 1:50 - 1:100 |
反応性: | Human, Mouse, Rat |
ポジティブサンプル: | HT-29, SW480, SW620 |
免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2). |
精製方法: | Affinity purification |
ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
アイソタイプ: | IgG |
順序: | MLAT RRLL GWSL PARV SVRF SGDT TAPK KTSF GSLK DEDR IFTN LYGR HDWR LKGS LSRG DWYK TKEI LLKG PDWI LGEI KTSG LRGR GGAG FPTG LKWS FMNK PSDG RPKY LVVN ADEG EPGT CKDR EILR HDPH KLLE GCLV GGRA MGAR AAYI YIRG EFYN EASN LQVA IREA YEAG LIGK NACG SGYD FDVF VVRG AGAY ICGE ETAL IESI EGKQ GKPR LKPP FPAD VGVF GCPT TVAN VETV AV |
遺伝子ID: | 4723 |
Uniprot: | P49821 |
セルラーロケーション: | Matrix side, Mitochondrion inner membrane, Peripheral membrane protein |
計算された分子量: | 49kDa/50kDa |
観察された分子量: | 40kDa |
同義語: | NDUFV1, CI-51K, CI51KD, UQOR1 |
バックグラウンド: | 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. |
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 |
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