Rabbit anti-Human, Mouse NUBPL Polyclonal Antibody | anti-NUBPL antibody

NUBPL, NT (NUBPL, C14orf127, Iron-sulfur protein NUBPL, IND1 homolog, Nucleotide-binding protein-like, huInd1) (FITC)

Cat. Num. AAA6327065

• Gene Names: NUBPL; IND1; huInd1; MC1DN21; C14orf127
• Reactivity: Human, Mouse
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: NUBPL; Polyclonal Antibody; NT (NUBPL; C14orf127; Iron-sulfur protein NUBPL; IND1 homolog; Nucleotide-binding protein-like; huInd1) (FITC); huInd1; anti-NUBPL antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
• Sequence Length: 319

• Applicable Applications for anti-NUBPL antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: NUBPL antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 39-67 amino acids from the N-terminal region of human NUBPL.
• Conjugate: FITC
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(NUBPL Antibody (N-term) western blot analysis in mouse bladder tissue lysates (35ug/lane).This demonstrates the NUBPL antibody detected the NUBPL protein (arrow).)

Product Categories/Family for anti-NUBPL antibody

Antibodies; Binding Proteins

References

Calvo, S.E., et al. Nat. Genet. 42(10):851-858(2010). Sheftel, A.D., et al. Mol. Cell. Biol. 29(22):6059-6073(2009). Volpi, S., et al. Mol. Psychiatry 14(11):1024-1031(2009). Baranzini, S.E., et al. Hum. Mol. Genet. 18(4):767-778(2009). Lamesch, P., et al. Genomics 89(3):307-315(2007).

NCBI and Uniprot Product Information

• NCBI GI #: 157384956
• NCBI GeneID: 80224
• NCBI Accession #: NP_079428.2
• NCBI GenBank Nucleotide #: NP_079428.2
• UniProt Accession #: Q8TB37
• NCBI Official Full Name: iron-sulfur protein NUBPL isoform 1
• NCBI Official Synonym Full Names: nucleotide binding protein like
• NCBI Official Symbol: NUBPL
• NCBI Official Synonym Symbols: IND1; huInd1; MC1DN21; C14orf127
• NCBI Protein Information: iron-sulfur protein NUBPL
• UniProt Protein Name: Iron-sulfur protein NUBPL
• Protein Family: Iron-sulfur protein
• UniProt Gene Name: NUBPL
• UniProt Synonym Gene Names: C14orf127
• UniProt Entry Name: NUBPL_HUMAN

NCBI Description

This gene encodes a member of the Mrp/NBP35 ATP-binding proteins family. The encoded protein is required for the assembly of the respiratory chain NADH dehydrogenase (complex I), an oligomeric enzymatic complex located in the inner mitochondrial membrane. Mutations in this gene cause mitochondrial complex I deficiency. Alternative splicing results in multiple transcript variants. [provided by RefSeq, May 2014]

Uniprot Description

NUBPL: Required for the assembly of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I). May deliver of one or more Fe-S clusters to complex I subunits. Defects in NUBPL are a cause of mitochondrial complex I deficiency (MT-C1D). A disorder of the mitochondrial respiratory chain that causes a wide range of clinical manifestations 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 Mrp/NBP35 ATP-binding proteins family. 2 isoforms of the human protein are produced by alternative splicing.

Chromosomal Location of Human Ortholog: 14q12

Cellular Component: mitochondrion

Molecular Function: 4 iron, 4 sulfur cluster binding; metal ion binding; ATP binding

Biological Process: mitochondrial respiratory chain complex I assembly

Disease: Mitochondrial Complex I Deficiency