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NUBPL Antibody (PACO38010)

SKU:
PACO38010
Product Type:
Antibody
Reactivity:
Human
Host Species:
Rabbit
Isotype:
IgG
Applications:
ELISA
WB
IHC
Antibody Type:
Polyclonal Antibody
Conjugation:
Unconjugated
  • NUBPL Antibody PACO38010
  • NUBPL Antibody PACO38010
  • NUBPL Antibody PACO38010
€359

Description

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NUBPL Antibody (PACO38010)

The NUBPL Polyclonal Antibody (PACO38010) is a valuable tool for researchers studying NUBPL, a gene involved in cellular energy production and metabolism. This antibody, generated in rabbits, is highly specific for human samples and is validated for use in applications such as Western blotting. By binding to the NUBPL protein, this antibody enables detailed analysis and detection in various cell types, making it ideal for investigations in the fields of biochemistry and mitochondrial biology.

NUBPL is a crucial component of the mitochondrial respiratory chain, playing a key role in the maintenance of cellular function and energy production. Mutations in the NUBPL gene have been linked to various mitochondrial disorders and neurological diseases, making it a promising target for therapeutic research. By studying the function and expression of NUBPL, researchers can gain insight into the mechanisms underlying these conditions and potentially develop new treatment strategies.

Antibody Name:NUBPL Antibody (PACO38010)
Antibody SKU:PACO38010
Size:50ug
Host Species:Rabbit
Tested Applications:ELISA, WB, IHC
Recommended Dilutions:ELISA:1:2000-1:10000, WB:1:500-1:2000, IHC:1:20-1:200
Species Reactivity:Human
Immunogen:Recombinant Human Iron-sulfur protein NUBPL protein (39-319AA)
Form:Liquid
Storage Buffer:Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
Purification Method:Antigen Affinity Purified
Clonality:Polyclonal
Isotype:IgG
Conjugate:Non-conjugated
Product ImageWestern blot. All lanes: NUBPL antibody at 1µg/ml + A431 whole cell lysate. Secondary. Goat polyclonal to rabbit IgG at 1/10000 dilution. Predicted band size: 35, 19 kDa. Observed band size: 35 kDa.
Product ImageImmunohistochemistry of paraffin-embedded human liver cancer using PACO38010 at dilution of 1:100.
Product ImageImmunohistochemistry of paraffin-embedded human colon cancer using PACO38010 at dilution of 1:100.
Background: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.
Synonyms:Iron-sulfur protein NUBPL (IND1 homolog) (Nucleotide-binding protein-like) (huInd1), NUBPL, C14orf127
UniProt Protein Function: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: 14q12Cellular Component: mitochondrial matrix; mitochondrionMolecular Function: 4 iron, 4 sulfur cluster bindingBiological Process: mitochondrial respiratory chain complex I assemblyDisease: Mitochondrial Complex I Deficiency
UniProt Protein Details:
NCBI Summary: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 Code:Q8TB37
NCBI GenInfo Identifier:116242683
NCBI Gene ID:80224
NCBI Accession:Q8TB37.3
UniProt Secondary Accession:Q8TB37,Q86TZ4, Q9H9M2, B4DHZ1
UniProt Related Accession:Q8TB37
Molecular Weight:18,209 Da
NCBI Full Name:Iron-sulfur protein NUBPL
NCBI 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
UniProt Synonym Protein Names:IND1 homolog; Nucleotide-binding protein-like; huInd1
Protein Family:Iron-sulfur protein
UniProt Gene Name:NUBPL  
UniProt Entry Name:NUBPL_HUMAN
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