Mucolipin 1 Antibody - Azide and BSA Free

Western Blot: Mucolipin 1 Antibody [H00057192-B01P] - Analysis of MCOLN1 expression in transfected 293T cell line by MCOLN1 polyclonal antibody. Lane 1: MCOLN1 transfected lysate(63.8 KDa). Lane 2: Non-transfected lysate.

• Reactivity: Hu
• Applications: WB
• Clonality: Polyclonal
• Host: Mouse
• Conjugate: Unconjugated
• Format: Azide and BSA Free

Mucolipin 1 Antibody - Azide and BSA Free Summary

• Description: Novus Biologicals Mouse Mucolipin 1 Antibody - Azide and BSA Free (H00057192-B01P) is a polyclonal antibody validated for use in WB. All Novus Biologicals antibodies are covered by our 100% guarantee.
• Immunogen: MCOLN1 (NP_065394.1, 1 a.a. - 580 a.a.) full-length human protein. MTAPAGPRGSETERLLTPNPGYGTQAGPSPAPPTPPEEEDLRRRLKYFFMSPCDKFRAKGRKPCKLMLQVVKILVVTVQLILFGLSNQLAVTFREENTIAFRHLFLLGYSDGADDTFAAYTREQLYQAIFHAVDQYLALPDVSLGRYAYVRGGGDPWTNGSGLALCQRYYHRGHVDPANDTFDIDPMVVTDCIQVDPPERPPPPPSDDLTLLESSSSYKNLTLKFHKLVNVTIHFRLKTINLQSLINNEIPDCYTFSVLITFDNKAHSGRIPISLETQAHIQECKHPSVFQHGDNSFRLLFDVVVILTCSLSFLLCARSLLRGFLLQNEFVGFMWRQRGRVISLWERLEFVNGWYILLVTSDVLTISGTIMKIGIEAKNLASYDVCSILLGTSTLLVWVGVIRYLTFFHNYNILIATLRVALPSVMRFCCCVAVIYLGYCFCGWIVLGPYHVKFRSLSMVSECLFSLINGDDMFVTFAAMQAQQGRSSLVWLFSQLYLYSFISLFIYMVLSLFIALITGAYDTIKHPGGAGAEESELQAYIAQCQDSPTSGKFRRGSGSACSLLCCCGRDPSEEHSLLVN
• Specificity: Reacts with mucolipin 1.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Mouse
• Gene: MCOLN1
• Purity: Protein A purified

Applications/Dilutions

• Dilutions:
  • Western Blot
• Application Notes: This antibody is reactive against transfected lysate in western blot, and as a detection antibody in ELISA.

Packaging, Storage & Formulations

• Storage: Aliquot and store at -20C or -80C. Avoid freeze-thaw cycles.
• Buffer: PBS (pH 7.4)
• Preservative: No Preservative
• Purity: Protein A purified

Notes

This product is produced by and distributed for Abnova, a company based in Taiwan.

Alternate Names for Mucolipin 1 Antibody - Azide and BSA Free

• MCOLN1
• MG-2
• ML1
• ML4
• ML4TRP-ML1
• MLIV
• MST080
• MSTP080
• Mucolipidin
• mucolipidosis type IV protein
• Mucolipin 1
• mucolipin-1
• TRPML1
• TRP-ML1
• TRPM-L1

Background

Mucolipin-1 (MCOLN1) is a 65kDa member of the TRPML channel family, which is a subgroup of the large protein family of TRP ion channels. Mucolipin 1 is thought to channel iron ions across the endosome/lysosome membrane into the cell, so defective MCOLN1 may cause cellular iron deficiency.

Defects in MCOLN1 are known to cause 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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Gene Symbol: MCOLN1
• Entrez:
  • 57192(Human)
• Uniprot:
  • NP_065394.1(Human)