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货号： bsm-33241M-AF488 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bsm-33241M-AF488
- 英文名称
- Anti-HSP22/AF488
- 中文名称
- AF488标记的热休克蛋白-22抗体
- 别 名
- CMT2L; CRYAC; DHMN 2; DHMN2; E2 induced gene 1 protein; E2IG1; H11; Heat shock 22kDa protein 8; Heat shock protein 22; Heat shock protein beta 8; Hereditary motor neuropathy distal; HMN 2; HMN2; HSB8; HSPB 8; HSPB8; Protein kinase H11; Small stress protein like protein HSP22; Spinal muscular atrophy distal adult autosomal dominant; Alpha crystallin C chain; Charcot Marie Tooth disease axonal type 2L; Charcot Marie Tooth disease spinal.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 免疫学 信号转导 转录调节因子
- 抗体来源
- Mouse
- 克隆类型
- Monoclonal
- 克 隆 号
- 4C3
- 交叉反应
- Human, Mouse, Rat,
- 产品应用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 22kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 2mg/1ml
- 免 疫 原
- Recombinant human HSP22 Protein
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein G
- 储 存 液
- Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 产品介绍
- background:
Hsp22 (HSPB8) is a 196-amino acid protein that contains a central portion homologous to a highly conserved HSP-alpha crystallin domain common to all the small heat shock protein (HSP20) family members. Hsp22 is a monomeric protein which interacts with HSPB1. It displays temperature-dependent chaperone activity. The highest abundance of Hsp22 is in skeletal muscle, heart, and placenta. Mutations in the HSPB8 gene are associated with the inherited peripheral neuropathies, autosomal dominant distal hereditary motor neuropathy type IIA (dSMA) and axonal Charcot-Marie-Tooth disease type 2L (CMT2L).
Function:
Displays temperature-dependent chaperone activity.
Subunit:
Monomer. Interacts with HSPB1. Interacts with DNAJB6.
Subcellular Location:
Cytoplasm. Nucleus. Note=Translocates to nuclear foci during heat shock.
Tissue Specificity:
Predominantly expressed in skeletal muscle and heart.
DISEASE:
Defects in HSPB8 are the cause of distal hereditary motor neuronopathy type 2A (HMN2A) [MIM:158590]; also known as distal hereditary motor neuropathy type IIA or spinal Charcot-Marie-Tooth disease IIA. Distal hereditary motor neuronopathies constitute a heterogeneous group of neuromuscular disorders caused by selective impairment of motor neurons in the anterior horn of the spinal cord, without sensory deficit in the posterior horn. The overall clinical picture consists of a classical distal muscular atrophy syndrome in the legs without clinical sensory loss. The disease starts with weakness and wasting of distal muscles of the anterior tibial and peroneal compartments of the legs. Later on, weakness and atrophy may expand to the proximal muscles of the lower limbs and/or to the distal upper limbs.
Defects in HSPB8 are the cause of Charcot-Marie-Tooth disease type 2L (CMT2L) [MIM:608673]. CMT2L is an axonal form of Charcot-Marie-Tooth disease. Axonal CMT neuropathies are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy.
Similarity:
Belongs to the small heat shock protein (HSP20) family.
Database links:Entrez Gene: 26353 Human
Entrez Gene: 80888 Mouse
Entrez Gene: 113906 Rat
Omim: 608014 Human
SwissProt: Q9UJY1 Human
SwissProt: Q9JK92 Mouse
SwissProt: Q9EPX0 Rat
Unigene: 400095 Human
Unigene: 21549 Mouse
Unigene: 102906 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.