视神经萎缩相关蛋白1抗体
规格：1mg/1ml
英文名： OPA1
别名： Dynamin like 120 kDa protein; Dynamin like 120 kDa protein, mitochondrial; Dynamin-like 120 kDa protein; Dynamin-like 120 kDa protein, form S1; FLJ12460; Juvenile kjer type optic atrophy; Juvenile kje
分子量： 111kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human OPA1
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞浆 细胞膜
视神经萎缩相关蛋白1抗体产品介绍：background: OPA1 is a 120kDa protein belonging to the dynamin family. The OPA1 gene has been localized to 3q29. The gene is targeted to mitochondria and is involved in mitochondrial biogenesis. Defects in OPA1 are a cause of optic atrophy type 1. OPA1 is mostly expressed in retina but can also be expressed in brain, testis, heart and skeletal muscle. Function: 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. Subcellular Location: Mitochondrion inner membrane. Mitochondrion 视神经萎缩相关蛋白1抗体intermembrane space. Tissue Specificity: Highly expressed in retina. Also expressed in brain, testis, heart and skeletal muscle. Isoform 1 expressed in retina, skeletal muscle, heart, lung, ovary, colon, thyroid gland, leukocytes and fetal brain. Isoform 2 expressed in colon, liver, kidney, thyroid gland and leukocytes. Low levels of all isoforms expressed in a variety of tissues. Post-translational modifications: PARL-dependent proteolytic processing releases an antiapoptotic soluble form not required for mitochondrial fusion. DISEASE: Defects in OPA1 are a cause of optic atrophy type 1 (OPA1) [MIM:165500]. OPA1 is a dominantly inherited optic neuropathy occurring in 1 in 50,000 individuals that features progressive 视神经萎缩相关蛋白1抗体loss in visual acuity leading, in many cases, to legal blindness. Defects in OPA1 are the cause of optic atrophy 1 with deafness (OPA1D) [MIM:125250]. Some individuals with mutations in OPA1 manifest also ophthalmoplegia and myopathy. Similarity: Belongs to the dynamin family. Database links: UniProtKB/Swiss-Prot: O60313.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
视神经萎缩相关蛋白1抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 细胞生物 神经生物学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid