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Product Details
H00004976-M05
Product Specifications
Species Reactivity
Human
Host/Isotype
Mouse
/ IgG1, kappa
Class
Monoclonal
Type
Antibody
Clone
1C10
Immunogen
OPA1 (NP_056375, 851 a.a. approximately 960 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Conjugate
Form
Liquid
Concentration
See Label
Purification
Affinity chromatography
Storage buffer
PBS, pH 7.4
Contains
no preservative
Storage conditions
-20° C, Avoid Freeze/Thaw Cycles
Shipping conditions
Ambient (domestic); Wet ice (international)
Product Specific Information
Sequence of this protein is as follows: NHCNLCRRGF YYYQRHFVDS ELECNDVVLF WRIQRMLAIT ANTLRQQLTN TEVRRLEKNV KEVLEDFAED GEKKIKLLTG KRVQLAEDLK KVREIQEKLD AFIEALHQEK
Target Information
OPA1 is a dynamin-related GTPase that is critical for the maintenance of mitochondrial morphology and mtDNA. The most commonly associated phenotype with OPA1 mutations is heterozygous optic atrophy, a heterozygous dominant trait that causes reduced visual clarity and sometimes blindness. The disease usually begins in childhood and increases in severity throughout the life of affected individuals. Usually, this phenotype is attributed to the degeneration of optic nerve fibers. Interestingly, the same type of nerve degeneration seems to be partially causative of certain schizophrenia characteristics. OPA1 dysfunction also seems to be implicated in this case; mitochondrial networks associated with critical nerves seem to link schizophrenia and OPA1. The dysfunction is associated with issues with apoptosis and normal cellular metabolic regulation, all regulated through OPA1.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: Dynamin-like GTPase OPA1, mitochondrial; dynamin-like guanosine triphosphatase; mitochondrial dynamin-like GTPase; optic atrophy 1 (autosomal dominant); Optic atrophy protein 1
Gene Aliases: BERHS; KIAA0567; largeG; MGM1; MTDPS14; NPG; NTG; OPA1
UniProt ID: (Human) O60313
Entrez Gene ID: (Human) 4976
mitochondrial genome maintenance
mitochondrial fission
apoptotic process
mitochondrion organization
inner mitochondrial membrane organization
visual perception
mitochondrial fusion
positive regulation of mitochondrial fusion
positive regulation of neuron maturation
axonal transport of mitochondrion
negative regulation of release of cytochrome c from mitochondria
cellular senescence
positive regulation of dendrite development
negative regulation of endoplasmic reticulum stress-induced intrinsic apoptotic signaling pathway
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