乙酰胆碱酯酶相关胶原蛋白多肽抗体
规格：1mg/1ml
英文名： COLQ
别名： Acetylcholinesterase-associated collagen; AChE Q subunit; asymmetric acetylcholinesterase; Collagen-like tail subunit (single strand of homotrimer) of asymmetric acetylcholinesterase; Colq; COLQ_HUMAN
分子量： 45kDa
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 COLQ
交叉反应：Human, Mouse, Rat, Pig, Horse, Rabbit,
细胞定位：
乙酰胆碱酯酶相关胶原蛋白多肽抗体产品介绍：background: This gene encodes the subunit of a collagen-like molecule associated with acetylcholinesterase in skeletal muscle. Each molecule is composed of three identical subunits. Each subunit contains a proline-rich attachment domain (PRAD) that binds an acetylcholinesterase tetramer to anchor the catalytic subunit of the enzyme to the basal lamina. Mutations in this gene are associated with endplate acetylcholinesterase deficiency. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] Function: Anchors the catalytic subunits of asymmetric AChE to the 乙酰胆碱酯酶相关胶原蛋白多肽抗体synaptic basal lamina. Subcellular Location: Cell junction; synapse. Tissue Specificity: Found at the end plate of skeletal muscle. Post-translational modifications: The triple-helical tail is stabilized by disulfide bonds at each end. DISEASE: Defects in COLQ are the cause of congenital myasthenic syndrome Engel type (CMSE) [MIM:603034]; also known as end-plate acetylcholinesterase deficiency or congenital myasthenic syndrome type IC (CMS-IC). CMSE is a rare autosomal recessive congenital myasthenic syndrome characterized by onset during 乙酰胆碱酯酶相关胶原蛋白多肽抗体childhood, generalized weakness, abnormal fatigability on exertion, refrectoriness to acetylcholinesterase drugs, decremental electromyographic response and morphological abnormalities of the neuromuscular junctions. Similarity: Belongs to the COLQ family. Contains 2 collagen-like domains. Gene ID: 8292 Database links: Entrez Gene: 8292 Human Omim: 603033 Human SwissProt: Q9Y215 Human Unigene: 146735 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
乙酰胆碱酯酶相关胶原蛋白多肽抗体产品应用：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：神经生物学 信号转导 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid