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品种反应性 主要应用 宿主 格式 抗体类型 Eq, H, M, R, B WB Rb Affinity Purified Polyclonal Antibody 描述 产品目录编号 ABN95 描述 Anti-OPA1 Antibody Alternate Names - Dynamin-like 120 kDa protein, form S1
- Dynamin-like 120 kDa protein, mitochondrial
- Optic atrophy protein 1
背景信息 OPA1 (optic atrophy 1) is a dynamin-related GTPase that plays a role in genome preservation and mitochondrial morphology. Localization of OPA1 is thought to be anchored to the inner membrane of the mitochondria. OPA1 is highly expressed in inner and outer retinal neurons and is important for normal mitochondrial function in those cells. Mutations in OPA1 are the major cause of autosomal dominant optic atrophy. Recent research has found that OPA1 also has the ability to protect neurons from excitotoxic injury and could be a potential clinical target to increase endurance of neurons post brain damage. 产品信息 格式 Affinity Purified 控制 - Human brain tissue lysate
演示 Purified rabbit polyclonal in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. 应用 应用 Detect OPA1 using this Anti-OPA1 Antibody validated for use in WB, IH(P). 主要应用 - Western Blotting
应用说明 Immunohistochemistry Analysis: A 1:1,000 dilution from a representative lot detected OPA1 in rat brain, rate cerebellum and rat midbrain tissues. 生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to human OPA1 at the mitochondrial intermembrane domain. 表位 Mitochondrial intermembrane domain 浓缩 Please refer to the Certificate of Analysis for the lot-specific concentration. 宿主 Rabbit 特异性 This antibody recognizes OPA1 at the mitochondrial intermembrane domain. 品种反应性 HorseHumanMouseRatBovine Species Reactivity Note Demonstrated to react with Human, Mouse, and Rat. Predicted to react with Bovine and Equine based on 100% sequence homology. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_056375
Entrez基因汇总 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]. 基因符号 - OPA1
纯化方法 Affinity purified UniProt编号 - O60313
UniProt汇总 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.
SUBUNIT STRUCTURE: Oligomeric complex consisting of membrane-bound and soluble forms of OPA1. Interacts with CHCHD3 and IMMT; these interactions occur preferentially with soluble OPA1 forms. Binds PARL (By similarity).
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Single-pass membrane protein. Mitochondrion 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.
PTM: PARL-dependent proteolytic processing releases an antiapoptotic soluble form not required for mitochondrial fusion.
INVOLVEMENT IN 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 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.
SEQUENCE SIMILARITIES: Belongs to the dynamin family.
SEQUENCE CAUTION: The sequence AF416919 differs from that shown. Reason:产品使用声明 质量保证 Evaluated by Western Blot in human brain tissue lysate.
Western Blot Analysis: 0.1 µg/mL of this antibody detected OPA1 on 10 µg of human brain tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at 2-8°C from date of receipt. 包装信息 数量 100 µg
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