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Invitrogen
TRPML1 Recombinant Rabbit Monoclonal Antibody (PSH09-01)
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TRPML1 Antibody (MA562892) in IHC (P)
Immunohistochemical analysis of Mucolipin-1 (TRPML1) on formalin-fixed paraffin-embedded mouse brain tissue.The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissue was blocked in 1% BSA for 20 minutes at room temperature, then probed with TRPML1 Recombinant Rabbit Monoclonal Antibody (PSH09-01) (Product # MA562892) at 1:1,000 dilution for 1... View More
产品信息
MA562892
产品规格
种属反应
Human,
Mouse,
Rat
宿主/亚型
Rabbit
/ IgG
Expression System
HEK293 cells
分类
Recombinant Monoclonal
类型
Antibody
克隆号
PSH09-01
抗原
Recombinant protein within human Mucolipin-1 aa 66-321.
偶联物
形式
Liquid
浓度
1 mg/mL
纯化类型
Protein A
保存液
PBS, pH 7.4, with 0.1% BSA, 40% glycerol
内含物
0.05% sodium azide
保存条件
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
运输条件
Ambient (domestic); Wet ice (international)
产品详细信息
Positive Control: SK-MEL-28 cell lysate, NCI-H226 cell lysate, TT cell lysate, Mouse brain tissue lysate, Rat brain tissue lysate, human brain tissue, mouse brain tissue, rat brain tissue.
Tissue Specificity: Low tissue specificity
Subcellular Location: Late endosome membrane, Lysosome membrane, Cytoplasmic vesicle membrane, Cell projection, phagocytic cup, Cytoplasmic vesicle, phagosome membrane, Cell membrane.
Sequence Similarities: 89.1% Mouse
Predicted band size: 65 kDa.
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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生物信息学
蛋白别名: MCLN1; MCOLN1 mucolipin 1; MG-2; MGC7172; ML1; Mucolipidin; mucolipidosis type IV protein; Mucolipin-1; Transient receptor potential channel mucolipin 1; Transient receptor potential-mucolipin 1; TRPML1
基因别名: 2210015I05Rik; MCOLN1; MG-2; ML4; MLIV; MST080; MSTP080; mucolipidin; TRP-ML1; TRPM-L1; TRPML1
UniProt ID: (Human) Q9GZU1, (Mouse) Q99J21
Entrez Gene ID: (Human) 57192, (Mouse) 94178, (Rat) 288371
