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Invitrogen
TRPML1 Recombinant Rabbit Monoclonal Antibody (PSH09-01)
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FIGURE: 1 / 6
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
Product Details
MA562892
Applications
Tested Dilution
Publications
Product Specifications
Species Reactivity
Human,
Mouse,
Rat
Host/Isotype
Rabbit
/ IgG
Expression System
HEK293 cells
Class
Recombinant Monoclonal
Type
Antibody
Clone
PSH09-01
Immunogen
Recombinant protein within human Mucolipin-1 aa 66-321.
Conjugate
Form
Liquid
Concentration
1 mg/mL
Purification
Protein A
Storage buffer
PBS, pH 7.4, with 0.1% BSA, 40% glycerol
Contains
0.05% sodium azide
Storage conditions
Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Shipping conditions
Ambient (domestic); Wet ice (international)
Product Specific Information
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.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: 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
Gene Aliases: 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
Performance Guarantee
If an Invitrogen™ antibody doesn't perform as described on our website or datasheet,we'll replace the product at no cost to you, or provide you with a credit for a future purchase.*
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