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微晶体蛋白:一种甲状腺激素结合蛋白。

µ-Crystallin: A thyroid hormone binding protein.

机构信息

Department of Physiology School of Medicine, University of Maryland, Baltimore, Baltimore, MD 21201.

出版信息

Endocr Regul. 2021 May 21;55(2):89-102. doi: 10.2478/enr-2021-0011.

Abstract

µ-Crystallin is a NADPH-regulated thyroid hormone binding protein encoded by the gene in humans. It is primarily expressed in the brain, muscle, prostate, and kidney, where it binds thyroid hormones, which regulate metabolism and thermogenesis. It also acts as a ketimine reductase in the lysine degradation pathway when it is not bound to thyroid hormone. Mutations in can result in non-syndromic deafness, while its aberrant expression, predominantly in the brain but also in other tissues, has been associated with psychiatric, neuromuscular, and inflammatory diseases. CRYM expression is highly variable in human skeletal muscle, with 15% of individuals expressing ≥13 fold more mRNA than the median level. Ablation of the gene in murine models results in the hypertrophy of fast twitch muscle fibers and an increase in fat mass of mice fed a high fat diet. Overexpression of in mice causes a shift in energy utilization away from glycolysis towards an increase in the catabolism of fat via β-oxidation, with commensurate changes of metabolically involved transcripts and proteins. The history, attributes, functions, and diseases associated with , an important modulator of metabolism, are reviewed.

摘要

微晶体蛋白是一种 NADPH 调节的甲状腺激素结合蛋白,由人类的 基因编码。它主要在大脑、肌肉、前列腺和肾脏中表达,在这些组织中结合甲状腺激素,调节代谢和产热。当它不与甲状腺激素结合时,它还作为赖氨酸降解途径中的酮还原酶发挥作用。 基因突变可导致非综合征性耳聋,而其异常表达,主要在大脑中,但也在其他组织中,与精神、神经肌肉和炎症性疾病有关。CRYM 在人类骨骼肌中的表达高度可变,有 15%的个体表达的 mRNA 比中位数水平高 13 倍以上。在小鼠模型中敲除 基因导致快速抽搐肌肉纤维肥大和高脂肪饮食喂养的小鼠脂肪量增加。在小鼠中过表达 导致能量利用从糖酵解向通过β-氧化增加脂肪分解的转变,伴随着代谢相关转录物和蛋白质的相应变化。本文综述了代谢重要调节剂 的历史、属性、功能和相关疾病。

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