热休克蛋白-22抗体
规格:1mg/1ml
英文名: HSP22
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;
分子量: 22kDa
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 HSP22(87
交叉反应:Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit,
细胞定位:细胞核 细胞浆
热休克蛋白-22抗体产品介绍: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 热休克蛋白-22抗体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) 热休克蛋白-22抗体[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. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
热休克蛋白-22抗体产品应用:WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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:肿瘤 **学 信号转导 转录调节因子
储存条件: Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源: Rabbit
外观: Lyophilized or Liquid