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Results: 1 to 20 of 86

1.

Proteolytic cleavage of the disease-related lysosomal membrane glycoprotein CLN7.

Steenhuis P, Froemming J, Reinheckel T, Storch S.

Biochim Biophys Acta. 2012 Oct;1822(10):1617-28. doi: 10.1016/j.bbadis.2012.05.015. Epub 2012 Jun 2.

PMID:
22668694
[PubMed - indexed for MEDLINE]
Free Article
2.

Lysosomal targeting of the CLN7 membrane glycoprotein and transport via the plasma membrane require a dileucine motif.

Steenhuis P, Herder S, Gelis S, Braulke T, Storch S.

Traffic. 2010 Jul 1;11(7):987-1000. doi: 10.1111/j.1600-0854.2010.01073.x. Epub 2010 Apr 16.

PMID:
20406422
[PubMed - indexed for MEDLINE]
3.

Expression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosis.

Sharifi A, Kousi M, Sagné C, Bellenchi GC, Morel L, Darmon M, Hulková H, Ruivo R, Debacker C, El Mestikawy S, Elleder M, Lehesjoki AE, Jalanko A, Gasnier B, Kyttälä A.

Hum Mol Genet. 2010 Nov 15;19(22):4497-514. doi: 10.1093/hmg/ddq381. Epub 2010 Sep 7.

PMID:
20826447
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

Disrupted in renal carcinoma 2 (DIRC2), a novel transporter of the lysosomal membrane, is proteolytically processed by cathepsin L.

Savalas LR, Gasnier B, Damme M, Lübke T, Wrocklage C, Debacker C, Jézégou A, Reinheckel T, Hasilik A, Saftig P, Schröder B.

Biochem J. 2011 Oct 1;439(1):113-28. doi: 10.1042/BJ20110166.

PMID:
21692750
[PubMed - indexed for MEDLINE]
Free Article
5.

Gene disruption of Mfsd8 in mice provides the first animal model for CLN7 disease.

Damme M, Brandenstein L, Fehr S, Jankowiak W, Bartsch U, Schweizer M, Hermans-Borgmeyer I, Storch S.

Neurobiol Dis. 2014 May;65:12-24. doi: 10.1016/j.nbd.2014.01.003. Epub 2014 Jan 11.

PMID:
24423645
[PubMed - indexed for MEDLINE]
6.

Mutations in CLN7/MFSD8 are a common cause of variant late-infantile neuronal ceroid lipofuscinosis.

Kousi M, Siintola E, Dvorakova L, Vlaskova H, Turnbull J, Topcu M, Yuksel D, Gokben S, Minassian BA, Elleder M, Mole SE, Lehesjoki AE.

Brain. 2009 Mar;132(Pt 3):810-9. doi: 10.1093/brain/awn366. Epub 2009 Feb 5.

PMID:
19201763
[PubMed - indexed for MEDLINE]
Free Article
7.

Proteolytic processing regulates Toll-like receptor 3 stability and endosomal localization.

Qi R, Singh D, Kao CC.

J Biol Chem. 2012 Sep 21;287(39):32617-29. Epub 2012 Aug 3.

PMID:
22865861
[PubMed - indexed for MEDLINE]
Free PMC Article
8.
9.

A critical histidine residue within LIMP-2 mediates pH sensitive binding to its ligand β-glucocerebrosidase.

Zachos C, Blanz J, Saftig P, Schwake M.

Traffic. 2012 Aug;13(8):1113-23. doi: 10.1111/j.1600-0854.2012.01372.x. Epub 2012 May 15.

PMID:
22537104
[PubMed - indexed for MEDLINE]
10.

Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A.

Heine C, Koch B, Storch S, Kohlschütter A, Palmer DN, Braulke T.

J Biol Chem. 2004 May 21;279(21):22347-52. Epub 2004 Mar 9.

PMID:
15010453
[PubMed - indexed for MEDLINE]
Free Article
12.

The neuronal ceroid lipofuscinosis protein CLN5: new insights into cellular maturation, transport, and consequences of mutations.

Schmiedt ML, Bessa C, Heine C, Ribeiro MG, Jalanko A, Kyttälä A.

Hum Mutat. 2010 Mar;31(3):356-65. doi: 10.1002/humu.21195.

PMID:
20052765
[PubMed - indexed for MEDLINE]
13.

Contribution of cathepsin L to secretome composition and cleavage pattern of mouse embryonic fibroblasts.

Tholen S, Biniossek ML, Gessler AL, Müller S, Weisser J, Kizhakkedathu JN, Reinheckel T, Schilling O.

Biol Chem. 2011 Nov;392(11):961-71. doi: 10.1515/BC.2011.162.

PMID:
21972973
[PubMed - indexed for MEDLINE]
14.

Mutations in MFSD8/CLN7 are a frequent cause of variant-late infantile neuronal ceroid lipofuscinosis.

Aiello C, Terracciano A, Simonati A, Discepoli G, Cannelli N, Claps D, Crow YJ, Bianchi M, Kitzmuller C, Longo D, Tavoni A, Franzoni E, Tessa A, Veneselli E, Boldrini R, Filocamo M, Williams RE, Bertini ES, Biancheri R, Carrozzo R, Mole SE, Santorelli FM.

Hum Mutat. 2009 Mar;30(3):E530-40. doi: 10.1002/humu.20975.

PMID:
19177532
[PubMed - indexed for MEDLINE]
15.

The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brain.

Holmberg V, Jalanko A, Isosomppi J, Fabritius AL, Peltonen L, Kopra O.

Neurobiol Dis. 2004 Jun;16(1):29-40.

PMID:
15207259
[PubMed - indexed for MEDLINE]
17.

Proteolytic cleavage of ricin A chain in endosomal vesicles. Evidence for the action of endosomal proteases at both neutral and acidic pH.

Blum JS, Fiani ML, Stahl PD.

J Biol Chem. 1991 Nov 25;266(33):22091-5.

PMID:
1939230
[PubMed - indexed for MEDLINE]
Free Article
18.

Analysis of the biogenesis of heparan sulfate acetyl-CoA:alpha-glucosaminide N-acetyltransferase provides insights into the mechanism underlying its complete deficiency in mucopolysaccharidosis IIIC.

Durand S, Feldhammer M, Bonneil E, Thibault P, Pshezhetsky AV.

J Biol Chem. 2010 Oct 8;285(41):31233-42. doi: 10.1074/jbc.M110.141150. Epub 2010 Jul 22.

PMID:
20650889
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Glucose regulates protein catabolism in ras-transformed fibroblasts through a lysosomal-dependent proteolytic pathway.

Tournu C, Obled A, Roux MP, Ferrara M, Omura S, Béchet DM.

Biochem J. 2001 Jul 1;357(Pt 1):255-61.

PMID:
11415457
[PubMed - indexed for MEDLINE]
Free PMC Article
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