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产品信息
H00004976-M05
产品规格
种属反应
Human
宿主/亚型
Mouse
/ IgG1, kappa
分类
Monoclonal
类型
Antibody
克隆号
1C10
抗原
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.
偶联物
形式
Liquid
浓度
See Label
纯化类型
Affinity chromatography
保存液
PBS, pH 7.4
内含物
no preservative
保存条件
-20° C, Avoid Freeze/Thaw Cycles
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Sequence of this protein is as follows: NHCNLCRRGF YYYQRHFVDS ELECNDVVLF WRIQRMLAIT ANTLRQQLTN TEVRRLEKNV KEVLEDFAED GEKKIKLLTG KRVQLAEDLK KVREIQEKLD AFIEALHQEK
靶标信息
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.
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生物信息学
蛋白别名: Dynamin-like GTPase OPA1, mitochondrial; dynamin-like guanosine triphosphatase; mitochondrial dynamin-like GTPase; optic atrophy 1 (autosomal dominant); Optic atrophy protein 1
基因别名: 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
