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Key Documents

M8072

Sigma-Aldrich

Anti-Mucolipin-1 antibody, Mouse monoclonal

clone MLN128, purified from hybridoma cell culture

Synonyme(s) :

Anti-MCOLN1, Anti-ML4, Anti-MLIV, Anti-MST080, Anti-MSTP080, Anti-Mucolipidin, Anti-TRP-ML1, Anti-TRPM-L1, Anti-TRPML1, Anti-Transient receptor potential cation channel, mucolipin subfamily, member 1

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About This Item

Code UNSPSC :
12352203
Nomenclature NACRES :
NA.41

Source biologique

mouse

Niveau de qualité

Conjugué

unconjugated

Forme d'anticorps

purified from hybridoma cell culture

Type de produit anticorps

primary antibodies

Clone

MLN128, monoclonal

Forme

buffered aqueous solution

Poids mol.

antigen ~110 kDa (additional bands may be observed)

Espèces réactives

human

Conditionnement

antibody small pack of 25 μL

Concentration

~2.0 mg/mL

Technique(s)

immunocytochemistry: suitable
indirect ELISA: suitable
western blot: 4-8 μg/mL using membrane fraction of HEK-293T expressing human mucolipin-1

Numéro d'accès UniProt

Conditions d'expédition

dry ice

Température de stockage

−20°C

Modification post-traductionnelle de la cible

unmodified

Informations sur le gène

human ... MCOLN1(57192)

Catégories apparentées

Description générale

Monoclonal Anti-Mucolipin-1 (mouse IgG1 isotype) is derived from the hybridoma MLN128 produced by the fusion of mouse myeloma cells and splenocytes from BALB/c mice immunized with a recombinant fusion protein corresponding to amino acid 1.Mucolipin-1 is also termed TRP-ML1, MLN1, ML1 mucolipidin. MLN1 shares significant sequence homology with the TRP superfamily of cation channels.
Mucolipin-1 (MCOLN1) is a member of transient receptor potential (TRP) protein family. It is a cation channel present on endosomes and lysosomes.

Application

Monoclonal Anti-Mucolipin-1 has been used in:
  • enzyme linked immunosorbent assay (ELISA)
  • immunoblotting
  • immunocytochemistry.

Actions biochimiques/physiologiques

Mucolipin-1 (MCOLN1) is involved in the regulation of lysosomal trafficking. It aids in the transport of Ca2+ into the cytosol from the lumen, in response to the changing levels of phosphatidylinositol-3, 5-bisphosphate. Mutations in the gene encoding MCOLN1 have been shown to be associated with mucolipidosis type IV.
Mutations in the MCOLN1 gene is implicated in Mucolipidosis type IV (MLIV) is an autosomal recessive, neurodegenerative disorder. Rather, MLIV pathophysiology has been linked to deficiency in membrane trafficking, and organelle dynamics in the late endocytic pathway. Specifically, MLIV cells have been shown to accumulate autophagosomes, due to increased de novo autophagosome formation and due to delayed fusion of autophagosomes with late endosomes/lysosomes.

Forme physique

Solution in 0.01 M phosphate buffered saline, pH 7.4, containing 15 mM sodium azide.

Clause de non-responsabilité

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

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Code de la classe de stockage

10 - Combustible liquids

Classe de danger pour l'eau (WGK)

WGK 1

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

Équipement de protection individuelle

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)


Certificats d'analyse (COA)

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Consulter la Bibliothèque de documents

Autophagic dysfunction in mucolipidosis type IV patients
Vergarajauregui S, et al.
Human Molecular Genetics, 17(17), 2723-2737 (2008)
Posttranslational cleavage and adaptor protein complex-dependent trafficking of mucolipin-1
Miedel MT, et al.
The Journal of Biological Chemistry, 281(18), 12751-12759 (2006)
Zhenxing Liu et al.
Biochemistry and cell biology = Biochimie et biologie cellulaire, 92(4), 279-286 (2014-06-25)
Lysosomotropic amines cause serious side effects such as cytoplasmic vacuolation and cell death. TRPML1 (also known as mucolipin1), a member of the transient receptor potential (TRP) protein family, may regulate fusion/fission of vesicles along the endocytic pathway and some aspects
Helen Waller-Evans et al.
Biochemical Society transactions, 43(3), 442-446 (2015-05-27)
TRPML1 is a ubiquitously expressed cation channel found on lysosomes and late endosomes. Mutations in TRPML1 cause mucolipidosis type IV and it has been implicated in Alzheimer's disease and HIV. However, the mechanisms by which TRPML1 activity is regulated are not
Wuyang Wang et al.
Proceedings of the National Academy of Sciences of the United States of America, 112(11), E1373-E1381 (2015-03-04)
Upon nutrient starvation, autophagy digests unwanted cellular components to generate catabolites that are required for housekeeping biosynthesis processes. A complete execution of autophagy demands an enhancement in lysosome function and biogenesis to match the increase in autophagosome formation. Here, we

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