NUBPL Antibody (PACO38010)
- SKU:
- PACO38010
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- WB
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| 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 |
 | Western 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. |
 | Immunohistochemistry of paraffin-embedded human liver cancer using PACO38010 at dilution of 1:100. |
 | Immunohistochemistry 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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