Thermo Fisher Scientific TRPML1 Polyclonal Antibody
Rabbit polyclonal antibody against mouse TRPML1 protein. Validated for Western blot at 1:200 dilution. Lyophilized form, reconstitute with 50 µL deionized water. For research use only, not for diagnostic procedures.
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Applications
- Western Blot (WB): Tested dilution 1:200
Product Specifications
| 항목 | 내용 |
|---|---|
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Peptide (C)GRRASETERLLTPN, corresponding to amino acid residues 6–19 of mouse TRPML1 |
| Conjugate | Unconjugated |
| Form | Lyophilized |
| Concentration | 0.8 mg/mL |
| Storage Conditions | -20°C |
| Shipping Conditions | Wet ice |
| RRID | AB_2736742 |
Product Specific Information
Product is shipped at room temperature as a lyophilized powder and should be stored at -20°C upon receipt.
Reconstitute using 50 µL deionized water.
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 observed in almost every cell type of affected patients. Most patients are unable to speak or walk independently and reach a maximal developmental level of 1–2 years.
All patients have constitutive achlorhydria 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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