Article

JIP4 deficiency causes a lysosomal storage disease arising from impaired cystine efflux

2025-06-09

Abstract excerpt

Lysosomes break down macromolecules, clear cellular waste and recycle nutrients such as cystine. We describe a novel mechanism whereby JIP4 regulates lysosomal cystine storage by controlling the abundance of cystinosin (CTNS), the transporter responsible for lysosomal cystine efflux. To this end, JIP4, previously characterized as a motor adaptor and kinase signaling scaffold, suppresses TMEM55B-dependent ubiquityl...

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Literature Corpus work: e8a1c72c-3153-596c-a356-9006c299db42
DOI: 10.1101/2025.06.06.657909

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