MPV17 Antibody (PACO10562)
- SKU:
- PACO10562
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- WB
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | MPV17 Antibody (PACO10562) |
| Antibody SKU: | PACO10562 |
| Size: | 50ul |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB, IHC |
| Recommended Dilutions: | |
| Species Reactivity: | Human, Mouse, Rat |
| Immunogen: | Human MPV17 |
| Form: | Liquid |
| Storage Buffer: | PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles. |
| Purification Method: | Antigen Affinity Purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
| Synonyms: | MpV17 mitochondrial inner membrane protein;MPV17;SYM1 ; |
| UniProt Protein Function: | MPV17: Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance. Defects in MPV17 are the cause of mitochondrial DNA depletion syndrome type 6 (MTDPS6). A disease characterized by infantile onset of progressive liver failure, often leading to death in the first year of life, peripheral neuropathy, corneal scarring, acral ulceration and osteomyelitis leading to autoamputation, cerebral leukoencephalopathy, failure to thrive, and recurrent metabolic acidosis with intercurrent infections. Belongs to the peroxisomal membrane protein PXMP2/4 family.Protein type: Membrane protein, integral; Membrane protein, multi-pass; MitochondrialChromosomal Location of Human Ortholog: 2p23.3Cellular Component: mitochondrion; mitochondrial inner membrane; integral to membrane; peroxisomeBiological Process: mitochondrial genome maintenance; homeostatic process; glomerular basement membrane development; inner ear developmentDisease: Mitochondrial Dna Depletion Syndrome 6 (hepatocerebral Type) |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a mitochondrial inner membrane protein that is implicated in the metabolism of reactive oxygen species. Mutations in this gene have been associated with the hepatocerebral form of mitochondrial DNA depletion syndrome (MDDS). [provided by RefSeq, Jul 2008] |
| UniProt Code: | P39210 |
| NCBI GenInfo Identifier: | 730059 |
| NCBI Gene ID: | 4358 |
| NCBI Accession: | P39210.1 |
| UniProt Secondary Accession: | P39210,Q53SY2, Q96B08, D6W555 |
| UniProt Related Accession: | P39210, AAB25210 |
| Molecular Weight: | 176 |
| NCBI Full Name: | Protein Mpv17 |
| NCBI Synonym Full Names: | MpV17 mitochondrial inner membrane protein |
| NCBI Official Symbol: | MPV17 |
| NCBI Official Synonym Symbols: | SYM1; MTDPS6 |
| NCBI Protein Information: | protein Mpv17; Mpv17, human homolog of glomerulosclerosis and nephrotic syndrome |
| UniProt Protein Name: | Protein Mpv17 |
| UniProt Synonym Protein Names: | |
| Protein Family: | Mpv17-like protein |
| UniProt Gene Name: | MPV17 |
| UniProt Entry Name: | MPV17_HUMAN |
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