Department of Biochemistry and Molecular Biophysics, Kansas State University, Manhattan, KS 66506, USA.
Department of Neuroscience, Istituto di Ricerche Farmacologiche Mario Negri IRCCS, 20156 Milan, Italy.
Biomolecules. 2021 Mar 10;11(3):408. doi: 10.3390/biom11030408.
Human tripartite motif family of proteins 32 (TRIM32) is a ubiquitous multifunctional protein that has demonstrated roles in differentiation, muscle physiology and regeneration, and tumor suppression. Mutations in TRIM32 result in two clinically diverse diseases. A mutation in the B-box domain gives rise to Bardet-Biedl syndrome (BBS), a disease whose clinical presentation shares no muscle pathology, while mutations in the NHL (NCL-1, HT2A, LIN-41) repeats of TRIM32 causes limb-girdle muscular dystrophy type 2H (LGMD2H). TRIM32 also functions as a tumor suppressor, but paradoxically is overexpressed in certain types of cancer. Recent evidence supports a role for TRIM32 in glycolytic-mediated cell growth, thus providing a possible mechanism for TRIM32 in the accumulation of cellular biomass during regeneration and tumorigenesis, including in vitro and in vivo approaches, to understand the broad spectrum of TRIM32 functions. A special emphasis is placed on the utility of the model, a unique system to study glycolysis and anabolic pathways that contribute to the growth and homeostasis of both normal and tumor tissues.
人类三联基序蛋白家族 32(TRIM32)是一种普遍存在的多功能蛋白,已被证明在分化、肌肉生理学和再生以及肿瘤抑制中发挥作用。TRIM32 的突变导致两种临床表现不同的疾病。B 盒结构域的突变导致 Bardet-Biedl 综合征(BBS),该病的临床表现与肌肉病理学无关,而 NHL(NCL-1、HT2A、LIN-41)重复结构域的突变导致肢带型肌营养不良 2H(LGMD2H)。TRIM32 还作为一种肿瘤抑制因子,但奇怪的是在某些类型的癌症中过度表达。最近的证据支持 TRIM32 在糖酵解介导的细胞生长中的作用,从而为 TRIM32 在再生和肿瘤发生过程中积累细胞生物量提供了一种可能的机制,包括体外和体内方法,以了解 TRIM32 的广泛功能。特别强调了模型的实用性,这是一个独特的系统,用于研究糖酵解和合成代谢途径,这些途径有助于正常和肿瘤组织的生长和稳态。