|Western Blot (WB)||0.1-0.5 µg/ml|
|Tested Species reactivity||Human, Mouse, Rat|
|Host / Isotype||Rabbit / IgG|
|Immunogen||Synthetic peptide corresponding to residues 500-580 of a C-terminal portion of mouse Mucolipin-1. [Swiss-Prot# Q99J21]|
|Purification||Antigen affinity chromatography|
|Storage buffer||PBS with 30% glycerol|
|Contains||0.1% sodium azide|
|Storage conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
The target sequence has 85% sequence homology with monkey and 78% sequence homology with bovine.
Suggested positive control: mouse brain protein.
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.
Protein Aliases: MCOLN1; MG-2; ML4; MSTP080; Mucolipidin; mucolipidosis type IV protein; Mucolipin-1; TRPML-1 anti; TRPML1
Gene Aliases: 2210015I05Rik; MCOLN1; MG-2; ML4; MLIV; MST080; MSTP080; mucolipidin; TRP-ML1; TRPM-L1; TRPML1
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