Anti-NDUFV1 항체 (CAB8014)

Antibody Name:	NDUFV1 Rabbit Polyclonal Antibody
Antibody SKU:	CAB8014
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	HT-29, SW480, SW620

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 1-250 of human NDUFV1 (NP_009034.2).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	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
Gene ID:	4723
Uniprot:	P49821
Cellular Location:	Matrix side, Mitochondrion inner membrane, Peripheral membrane protein
Calculated MW:	49kDa/50kDa
Observed MW:	40kDa

Synonyms:

NDUFV1, CI-51K, CI51KD, UQOR1

Background:

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

	Western blot analysis of extracts of various cell lines, using NDUFV1 antibody (CAB8014) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
	Immunofluorescence analysis of A549 cells using NDUFV1 antibody (CAB8014). Blue: DAPI for nuclear staining.

Antibodies

Anti-NDUFV1 Antibody (CAB13333)

NDUFV1 Antibody (PACO10799)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)