OPA1 blocking peptide

OPA1

Cat. Num. AAA556174

• Gene Names: OPA1; NPG; NTG; MGM1; BERHS; largeG; MTDPS14
• Synonyms: OPA1; OPA1 blocking peptide

• Form/Format: 50ul liquid. Dissolved in DH20.
• Concentration: 1mg/ml (varies by lot)

• Peptide Sequence: A synthetic peptide made to an internal region within residues 500-600 of the human protein.
• Preparation and Storage: Storeat -20 degree C for shortterm and -80 degree C for long term. Avoid freeze-thaw cycles.

Testing Data

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Testing Data

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Related Product Information for OPA1 blocking peptide

OPA1 Blocking Peptide; specific to RA25019.
Control Antigen for use in absorption control.

This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Eight transcript variants encoding different isoforms, resulting from alternative splicing of exon 4 and two novel exons named 4b and 5b, have been reported for this gene.

NCBI and Uniprot Product Information

• NCBI GI #: 224831243
• NCBI GeneID: 4976
• NCBI Accession #: NP_056375.2
• NCBI GenBank Nucleotide #: NM_015560.2
• UniProt Accession #: O60313; D3DNW4
• Molecular Weight: 115,884 Da
• NCBI Official Full Name: dynamin-like 120 kDa protein, mitochondrial isoform 1
• NCBI Official Synonym Full Names: OPA1, mitochondrial dynamin like GTPase
• NCBI Official Symbol: OPA1
• NCBI Official Synonym Symbols: NPG; NTG; MGM1; BERHS; largeG; MTDPS14
• NCBI Protein Information: dynamin-like 120 kDa protein, mitochondrial
• UniProt Protein Name: Dynamin-like 120 kDa protein, mitochondrial
• Protein Family: Dynamin-like 120 kDa protein
• UniProt Gene Name: OPA1
• UniProt Synonym Gene Names: KIAA0567
• UniProt Entry Name: OPA1_HUMAN

NCBI Description

This gene product is a nuclear-encoded mitochondrial protein with similarity to dynamin-related GTPases. It is a component of the mitochondrial network. Mutations in this gene have been associated with optic atrophy type 1, which is a dominantly inherited optic neuropathy resulting in progressive loss of visual acuity, leading in many cases to legal blindness. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2009]

Uniprot Description

OPA1: a dynamin-related GTPase required for mitochondrial fusion and regulation of apoptosis. May form a diffusion barrier for proteins stored in mitochondrial cristae. Proteolytic processing in response to intrinsic apoptotic signals may lead to disassembly of OPA1 oligomers and release of the caspase activator cytochrome C (CYCS) into the mitochondrial intermembrane space. Defects in OPA1 are the cause of optic atrophy 1 and deafness. Eight splice-variant isoforms have been described.

Protein type: Membrane protein, integral; Hydrolase; Mitochondrial; Apoptosis; EC 3.6.5.5

Chromosomal Location of Human Ortholog: 3q29

Cellular Component: cytoplasm; dendrite; integral to membrane; membrane; mitochondrial crista; mitochondrial inner membrane; mitochondrial intermembrane space; mitochondrial outer membrane; mitochondrion; nucleoplasm

Molecular Function: GTP binding; GTPase activity; magnesium ion binding; protein binding

Biological Process: apoptosis; axon transport of mitochondrion; inner mitochondrial membrane organization and biogenesis; metabolic process; mitochondrial fission; mitochondrial fusion; mitochondrial genome maintenance; mitochondrion organization and biogenesis; positive regulation of neuron maturation; visual perception

Disease: Glaucoma, Normal Tension, Susceptibility To; Optic Atrophy 1; Optic Atrophy With Or Without Deafness, Ophthalmoplegia, Myopathy, Ataxia, And Neuropathy