Rabbit Anti-NDUFV1/FITC Conjugated antibody

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CI 51kD; Complex I 51kD; NADH dehydrogenase (ubiquinone) flavoprotein 1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial; NADH dehydrogenase flavoprotein 1; NADH ubiquinone oxidoreductase 51 kDa subunit; NADH ubiquinone oxidoreductase; NDUFV

Cat:

SL3959R-FITC

Species Reactivity：

Human,Mouse,Rat,(predicted: Dog,Pig,Cow,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human NDUFV1

Format：

Lyophilized or Liquid

Storage instructions：

Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

49kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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.[provided by RefSeq, Oct 2009]

Function:
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).

Subunit:
Complex I is composed of 45 different subunits. This is a component of the flavoprotein-sulfur (FP) fragment of the enzyme.

Subcellular Location:
Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.

DISEASE:
Defects in NDUFV1 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.

Similarity:
Belongs to the complex I 51 kDa subunit family.

Database links:

Entrez Gene: 287014 Cow

Entrez Gene: 4723 Human

Entrez Gene: 17995 Mouse

Entrez Gene: 293655 Rat

Omim: 161015 Human

SwissProt: P25708 Cow

SwissProt: P49821 Human

SwissProt: Q91YT0 Mouse

SwissProt: Q5XIH3 Rat

Unigene: 7744 Human

Unigene: 29842 Mouse

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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