Thermo Fisher Scientific TRPML1 Polyclonal Antibody
Human TRPML1 단백질을 인식하는 Rabbit Polyclonal Antibody로, WB 및 ICC/IF에 적합. 항원 친화 크로마토그래피로 정제되어 높은 특이성과 재현성을 제공. 연구용으로만 사용 가능하며, MLIV 관련 연구에 유용.
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Thermo Fisher Scientific TRPML1 Polyclonal Antibody
Applications and Tested Dilution
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 0.04–0.4 µg/mL |
| Immunocytochemistry (ICC/IF) | 0.25–2 µg/mL |
Product Specifications
| 항목 | 내용 |
|---|---|
| Species Reactivity | Human |
| Host / Isotype | Rabbit / IgG |
| Class | Polyclonal |
| Type | Antibody |
| Immunogen | Recombinant Protein corresponding to Human TRPML1. Recombinant protein control fragment (Product #RP-95585). |
| Conjugate | Unconjugated |
| Form | Liquid |
| Concentration | 0.08 mg/mL |
| Purification | Antigen affinity chromatography |
| Storage Buffer | PBS, pH 7.2, with 40% glycerol |
| Contains | 0.02% sodium azide |
| Storage Conditions | Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles. |
| Shipping Conditions | Wet ice |
| RRID | AB_2856440 |
Product Specific Information
Immunogen sequence:
DTIKHPGGAG AEESELQAYI AQCQDSPTSG KFRRGSGSAC SLLCCCGRDP SE
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
- Ophthalmologic abnormalities (corneal opacity, retinal degeneration, strabismus)
- Storage bodies of lipids and water-soluble substances visible by electron microscopy
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 elevated serum gastrin levels.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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