Lysosomal pathology and osteopetrosis upon loss of H+-driven lysosomal Cl- accumulation

Science

Volumn 328, Issue 5984, 2010, Pages 1401-1403

  Weinert, Stefanie ^a,b   Jabs, Sabrina ^a,b,f   Supanchart, Chayarop ^c   Schweizer, Michaela ^d   Gimber, Nielas ^a,b   Richter, Martin ^a,f   Rademann, Jörg ^a,f,g   Stauber, Tobias ^a,b   Kornak, Uwe ^c,e   Jentsch, Thomas J ^a,b  

^a Leibniz Institut für Molekular Pharmakologie FMP   (Germany)

^b MAX DELBRÜCK CENTER FOR MOLECULAR MEDICINE   (Germany)

^c CHARITÉ UNIVERSITÄTSMEDIZIN BERLIN   (Germany)

^d UNIVERSITY OF HAMBURG   (Germany)

^e MAX PLANCK INSTITUTE FOR MOLECULAR GENETICS   (Germany)

^f FREIE UNIVERSITÄT BERLIN   (Germany)

^g UNIVERSITY OF LEIPZIG   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CHLORIDE; CHLORIDE CHANNEL; PROTEIN CLC 7; PROTON; PROTON PUMP; UNCLASSIFIED DRUG;

BIOACCUMULATION; ELECTRON; ION EXCHANGE; MUTATION; PH; PHENOTYPE; RODENT;

ACIDIFICATION; ALBERS SCHOENBERG DISEASE; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; BIOACCUMULATION; CELL PH; CHLORIDE CONDUCTANCE; CHLORIDE PROTON EXCHANGE; CONTROLLED STUDY; LYSOSOME; LYSOSOME STORAGE DISEASE; MOUSE; NONHUMAN; PHENOTYPE; POINT MUTATION; PRIORITY JOURNAL; PROTON TRANSPORT; WILD TYPE;

MUS;

EID: 77953564940     PISSN: 00368075     EISSN: 10959203     Source Type: Journal    
DOI: 10.1126/science.1188072     Document Type: Article

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24. We thank I. Freyert, A. von Bock, and P. Göntz for technical assistance; M. Falcke for help with modeling; and S. Grinstein for suggesting protonophore pretreatment of lysosomes. Support was provided by the Deutsche Forschungsgemeinschaft [grants Je164/7 and SFB740 (C5)] to T.J.J., SFB765(Ra895/6) to J.R., and the OSTEOPETR E-Rare grant (Bundesministerium für Bildung und Forschung) to UK