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Invitrogen
TRPML1 Polyclonal Antibody
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产品信息
PA5-77337
产品规格
宿主/亚型
Rabbit
/ IgG
分类
Polyclonal
类型
Antibody
抗原
Peptide (C)GRRASETERLLTPN, corresponding to amino acid residues 6-19 of mouse TRPLM1
偶联物
形式
Lyophilized
浓度
0.8 mg/mL
保存条件
-20°C
运输条件
Wet ice
RRID
产品详细信息
Product is shipped at room temperature as a lyophilized powder and should be stored at -20C upon receipt.
Reconstitute using 50 uL deionized water.
靶标信息
Defects in Mucolipin-1 are the cause of mucolipidosis type IV (MLIV), also known as sialolipidosis. MLIV is an autosomal recessive lysosomal storage disorder characterized by severe psychomotor retardation and ophthalmologic abnormalities, including corneal opacity, retinal degeneration and strabismus. Storage bodies of lipids and water-soluble substances are seen by electron microscopy in almost every cell type of the patients. Most patients are unable to speak or walk independently and reach a maximal developmental level of 1-2 years. All patients have constitutive achlorhydia associated with a secondary elevation of serum gastrin levels.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
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