Invitrogen™ TRPML1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Brand:  Invitrogen™ PA5111029

Product Code. 16803343

Description

Immunogen sequence: DTIKHPGGAG AEESELQAYI AQCQDSPTSG KFRRGSGSAC SLLCCCGRDP SE Highest antigen sequence indentity to the following orthologs: Mouse - 87%, Rat - 85%.

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.

Specifications

• Antigen: TRPML1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: MCOLN1
• Gene Alias: 2210015I05Rik; MCLN1; Mcoln1; MCOLN1 mucolipin 1; MG-2; MGC7172; ML1; ML4; MLIV; MST080; MSTP080; Mucolipidin; mucolipidosis type IV protein; mucolipin 1; mucolipin-1; Transient receptor potential channel mucolipin 1; Transient receptor potential-mucolipin 1; TRPML1; TRP-ML1; TRPM-L1
• Host Species: Rabbit
• Purification Method: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 57192
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Western Blot, Immunocytochemistry
• Concentration: 0.1 mg/mL
• Formulation: PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
• Gene Accession No.: Q9GZU1
• Gene Symbols: MCOLN1
• Immunogen: Recombinant Protein corresonding to Human TRPML1. Recombinant protein control fragment (Product #RP-95585).
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG