产品详情
英文名称 Dymeclin
中文名称 迪格弗-梅尔基奥尔-克劳森综合征相关蛋白抗体
别 名 DMC; Dyggve-Melchior-Clausen syndrome protein; DYM; FLJ20071; FLJ90130; SMC; DYM_HUMAN.
说 明 书 0.2ml
研究领域 细胞生物 发育生物学 信号转导
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Sheep,
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 迪格弗-梅尔基奥尔-克劳森综合征相关蛋白抗体(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.
分 子 量 76kDa
细胞定位 细胞浆
性 状 Lyophilized or Liquid
浓 度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human Dymeclin
亚 型 IgG
纯化方法 affinity purified by Protein A
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.
迪格弗-梅尔基奥尔-克劳森综合征相关蛋白抗体产品介绍 background:
Dyggve-Melchior-Clausen syndrome (DMC), a rare autosomal recessive disorder, is characterized by microcephaly, short trunk dwarfism and sometime psychomotor retardation. Cutaneous cells of affected individuals show dilated rough endoplasmic reticulum and enlarged vacuoles. The Dyggve-Melchior-Clausen syndrome protein, also designated dymeclin, may play a role in proteoglycan metabolism and intracellular protein digestion. It is a widely expressed multi-pass membrane protein, detected primarily in chondrocytes and fetal brain tissue. Defects in dymeclin are also the cause of Smith-McCort dysplasis syndrome (SMC), which has characteristics identical to those of Dyggve-Melchior-Clausen syndrome.
Function:
Necessary for correct organization of Golgi apparatus. Involved in bone development.
Subunit:
Interacts with GOLM1 and PPIB.
Subcellular Location:
Cytoplasmic and Golgi Apparatus
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