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Disease-associated Ca<jats:sub>V<\/jats:sub>2.1 mutants may exhibit defective proteostasis and promote endoplasmic reticulum (ER)-associated degradation of their wild-type (WT) counterpart in a dominant-negative manner. The E3 ubiquitin ligase RNF138 was previously shown to mediate EA2-related aberrant degradation of Ca<jats:sub>V<\/jats:sub>2.1 at the ER. Herein we aimed to elucidate the ER proteostasis mechanism of Ca<jats:sub>V<\/jats:sub>2.1. The peptidyl-prolyl <jats:italic>cis\/trans<\/jats:italic> isomerase, NIMA-interacting 1 (Pin1) was identified as a novel neuronal Ca<jats:sub>V<\/jats:sub>2.1 binding partner that promoted polyubiquitination and proteasomal degradation of Ca<jats:sub>V<\/jats:sub>2.1. Suppression of endogenous Pin1 level with either shRNA knockdown or the Pin1 inhibitor all-<jats:italic>trans<\/jats:italic> retinoic acid (ATRA) enhanced endogenous Ca<jats:sub>V<\/jats:sub>2.1 protein level in neurons, and attenuated ER-associated degradation of Ca<jats:sub>V<\/jats:sub>2.1 WT and EA2-causing mutants. Detailed mutation analyses suggested that Pin1 interacted with specific phosphorylated serine\/threonine-proline motifs in the intracellular II-III loop and the distal carboxy-terminal region of human Ca<jats:sub>V<\/jats:sub>2.1. We further generated Pin1-insensitive Ca<jats:sub>V<\/jats:sub>2.1 constructs and demonstrated that, during ER quality control, Pin1 served as an upstream regulator of Ca<jats:sub>V<\/jats:sub>2.1 polyubiquitination and degradation by RNF138. Pin1 regulation was required for the dominant-negative effect of EA2 missense mutants, but not nonsense mutants, on Ca<jats:sub>V<\/jats:sub>2.1 WT protein expression. Our data are consistent with the idea that Ca<jats:sub>V<\/jats:sub>2.1 proteostasis at the ER, as well as dominant-negative suppression of disease-causing loss-of-function mutants on Ca<jats:sub>V<\/jats:sub>2.1 WT, entail both Pin1\/RNF138-dependent and -independent mechanisms.<\/jats:p>","DOI":"10.1186\/s12964-024-01960-9","type":"journal-article","created":{"date-parts":[[2024,11,28]],"date-time":"2024-11-28T09:10:21Z","timestamp":1732785021000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Pin1 promotes human CaV2.1 channel polyubiquitination by RNF138: pathophysiological implication for episodic ataxia type 2"],"prefix":"10.1186","volume":"22","author":[{"given":"Ssu-Ju","family":"Fu","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kai-Min","family":"Cheng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cheng-Tsung","family":"Hsiao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ya-Ching","family":"Fang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chung-Jiuan","family":"Jeng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chih-Yung","family":"Tang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,11,28]]},"reference":[{"key":"1960_CR1","first-page":"398","volume":"20","author":"H Abriel","year":"2005","unstructured":"Abriel H, Staub O. 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