Mouse NUBPL Monoclonal Antibody | anti-NUBPL antibody

NUBPL antibody

Cat. Num. AAA833371

• Gene Names: NUBPL; IND1; huInd1; C14orf127
• Applications: Flow Cytometry, Functional Assay, Western Blot
• Purity: NUBPL antibody was purified by affinity chromatography.
• Synonyms: NUBPL; Monoclonal Antibody; NUBPL antibody; Monoclonal NUBPL; Anti-NUBPL; Nucleotide binding protein-like; HuInd1; IND1; FLJ12660; Iron-sulfur protein NUBPL; Chromosome 14 open reading frame 127; anti-NUBPL antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG1
• Clone Number: 2G7
• Purity/Purification: NUBPL antibody was purified by affinity chromatography.
• Form/Format: Supplied in PBS buffer, pH 7.3, containing 1% BSA, 50% glycerol and 0.02% sodium azide.
• Concentration: 500 ug-1 mg/ml (varies by lot)
• Sequence Length: 319

• Applicable Applications for anti-NUBPL antibody: Flow Cytometry (FC/FACS), Western Blot (WB)
• Application Notes: FC: 1:100; WB: 1:2000
• Biological Significance: NUBPL is 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 innermitochondrial membrane. The respiratory complex I consists of 45 subunits and 8 iron-sulfur (Fe/S) clusters. this protein is an Fe/S protein that plays a critical role in the assembly of respiratory complex I, likely by transferringFe/S into the Fe/S-containing complex I subunits.
• Cross-Reactivity: Human
• Immunogen: NUBPL antibody was raised in mouse using a human recombinant protein fragment corresponding to amino acids 1-250 of human NUBPL (NP_079428) produced in E Coli as the immunogen.
• Preparation and Storage: Store at 4 degree C for short term storage. Aliquot and store at -20 degree C for long term storage. Avoid repeated freeze/thaw cycles.

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of HEK293T cells transfected with either recombinant NUBPL protein (red) or empty vector (blue) stained using NUBPL antibody)

Western Blot (WB)

(Western Blot analysis of HEK293T cell lysates (5 ug) transfected with either recombinant NUBPL protein (Right) or empty vector (Left) detected with NUBPL antibody)

Related Product Information for anti-NUBPL antibody

Mouse monoclonal NUBPL antibody

Product Categories/Family for anti-NUBPL antibody

Cell Biology

NCBI and Uniprot Product Information

• NCBI GI #: 157384956
• NCBI GeneID: 80224
• NCBI Accession #: NP_079428
• NCBI GenBank Nucleotide #: NM_025152.2
• UniProt Accession #: Q8TB37; Q86TZ4; Q9H9M2; B4DHZ1
• Molecular Weight: 18,209 Da
• 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; 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: metal ion binding; 4 iron, 4 sulfur cluster binding; ATP binding

Biological Process: mitochondrial respiratory chain complex I assembly

Disease: Mitochondrial Complex I Deficiency