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Wani A, Weihl CC. Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP. Autophagy. 2021;17(12):4502-4503doi: 10.1080/15548627.2021.1985880.
Wani, A., & Weihl, C. C. (2021). Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP. Autophagy, 17(12), 4502-4503. https://doi.org/10.1080/15548627.2021.1985880
Wani, Abubakar, and Weihl, Conrad C. "Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP." Autophagy vol. 17,12 (2021): 4502-4503. doi: https://doi.org/10.1080/15548627.2021.1985880
Wani A, Weihl CC. Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP. Autophagy. 2021 Dec;17(12):4502-4503. doi: 10.1080/15548627.2021.1985880. Epub 2021 Oct 10. PMID: 34632910.
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Autophagy. 2021 Dec;17(12):4502-4503. doi: 10.1080/15548627.2021.1985880. Epub 2021 Oct 10.

Loss-of-function mutation in VCP mimics the characteristic pathology as in FTLD-TARDBP.

Autophagy

Abubakar Wani, Conrad C Weihl

Affiliations

  1. Department of Neurology, Hope Center for Neurological Diseases, Washington University School of Medicine, St. Louis, MO, USA.
  2. Department of Immunology, St. Jude Children's Research Hospital, Memphis, TN, USA.

PMID: 34632910 DOI: 10.1080/15548627.2021.1985880

Abstract

VCP (valosin containing protein), a member of the AAA+ protein family, is critical for many cellular processes and functions. Dominant VCP mutations cause a rare neurodegenerative disease known as multisystem proteinopathy (MSP). The spectrum of mechanisms causing fronto-temporal dementia with TARDBP/TDP-43 inclusions (FTLD-TARDBP) by VCP disease mutations remains unclear. Our recent work identified VCP activity as a mediator of FTLD-TARDBP. Specifically, brain atrophy, behavioral changes, neuronal loss, gliosis, and TARDBP pathology were observed in

Keywords: Autophagy; FTLD-TDP-43; neurodegeneration; progranulin; valosin-containing protein

References

  1. Cell Rep. 2021 Jul 20;36(3):109399 - PubMed

Publication Types

Grant support