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VCP/p97 cooperates with YOD1, UBXD1 and PLAA to drive clearance of ruptured lysosomes by autophagy.
Papadopoulos C, Kirchner P, Bug M, Grum D, Koerver L, Schulze N, Poehler R, Dressler A, Fengler S, Arhzaouy K, Lux V, Ehrmann M, Weihl CC, Meyer H. Papadopoulos C, et al. Among authors: weihl cc. EMBO J. 2017 Jan 17;36(2):135-150. doi: 10.15252/embj.201695148. Epub 2016 Oct 17. EMBO J. 2017. PMID: 27753622 Free PMC article.
Upon damage, p97 translocates to lysosomes and there cooperates with a distinct set of cofactors including UBXD1, PLAA, and the deubiquitinating enzyme YOD1, which we term ELDR components for Endo-Lysosomal Damage Response. Together, they act downstream of K63-linked ubiqu …
Upon damage, p97 translocates to lysosomes and there cooperates with a distinct set of cofactors including UBXD1, PLAA, and the deubiquitina …
Rapamycin nanoparticles target defective autophagy in muscular dystrophy to enhance both strength and cardiac function.
Bibee KP, Cheng YJ, Ching JK, Marsh JN, Li AJ, Keeling RM, Connolly AM, Golumbek PT, Myerson JW, Hu G, Chen J, Shannon WD, Lanza GM, Weihl CC, Wickline SA. Bibee KP, et al. Among authors: weihl cc. FASEB J. 2014 May;28(5):2047-61. doi: 10.1096/fj.13-237388. Epub 2014 Feb 5. FASEB J. 2014. PMID: 24500923 Free PMC article.
Current supportive therapy with corticosteroids results in a modest increase in strength as a consequence of a general reduction in inflammation, albeit with potential untoward long-term side effects and ultimate failure of the agent to maintain strength. ...
Current supportive therapy with corticosteroids results in a modest increase in strength as a consequence of a general reduction in inflamma …
Novel VCP mutations in inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia.
Watts GD, Thomasova D, Ramdeen SK, Fulchiero EC, Mehta SG, Drachman DA, Weihl CC, Jamrozik Z, Kwiecinski H, Kaminska A, Kimonis VE. Watts GD, et al. Among authors: weihl cc. Clin Genet. 2007 Nov;72(5):420-6. doi: 10.1111/j.1399-0004.2007.00887.x. Clin Genet. 2007. PMID: 17935506
Frontotemporal dementia associated with characteristic behavioral changes including short-term memory loss, language difficulty, and antisocial behavior was observed in three individuals at a mean age of 47 years. ...
Frontotemporal dementia associated with characteristic behavioral changes including short-term memory loss, language difficulty, and …