MPV17 blocking peptide

MPV17 Antibody (N-term) Blocking Peptide

• Gene Names: MPV17; SYM1; MTDPS6
• Synonyms: MPV17; MPV17 Antibody (N-term) Blocking Peptide; Protein Mpv17; MPV17 blocking peptide

• Specificity: The synthetic peptide sequence used to generate the antibody was selected from the N-term region of human MPV17. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.
• Form/Format: Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
• Sequence Length: 176

• Cellular Location: Mitochondrion inner membrane; Multi- pass membrane protein
• Tissue Location: Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.

Related Product Information for MPV17 blocking peptide

Involved in mitochondria homeostasis. May be involved in the metabolism of reactive oxygen species and control of oxidative phosphorylation and mitochondrial DNA (mtDNA) maintenance.

NCBI and Uniprot Product Information

• NCBI GI #: 730059
• NCBI GeneID: 4358
• UniProt Accession #: P39210; Q53SY2; Q96B08; D6W555; AAB25210
• Molecular Weight: 19,733 Da
• NCBI Official Full Name: Protein Mpv17
• NCBI Official Synonym Full Names: MPV17, mitochondrial inner membrane protein
• NCBI Official Symbol: MPV17
• NCBI Official Synonym Symbols: SYM1; MTDPS6
• NCBI Protein Information: protein Mpv17
• UniProt Protein Name: Protein Mpv17
• Protein Family: Mpv17-like protein
• UniProt Gene Name: MPV17
• UniProt Entry Name: MPV17_HUMAN

NCBI Description

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 Description

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; Mitochondrial

Chromosomal Location of Human Ortholog: 2p23.3

Cellular Component: mitochondrial inner membrane; mitochondrion; peroxisome

Biological Process: glomerular basement membrane development; homeostatic process; inner ear development; mitochondrial genome maintenance

Disease: Mitochondrial Dna Depletion Syndrome 6 (hepatocerebral Type)