| Literature DB >> 28577926 |
Lifang Hu1, Yunyun Xiao1, Zhipeng Xiong1, Fan Zhao1, Chong Yin1, Yan Zhang1, Peihong Su1, Dijie Li1, Zhihao Chen1, Xiaoli Ma1, Ge Zhang2, Airong Qian3.
Abstract
Spectraplakins are a family of evolutionarily conserved gigantic proteins and play critical roles in many cytoskeleton-related processes. Microtubule actin crosslinking factor 1 (MACF1) is one of the most versatile spectraplakin with multiple isoforms. As a broadly expressed mammalian spectraplakin, MACF1 is important in maintaining normal functions of many tissues. The loss-of-function studies using knockout mouse models reveal the pivotal roles of MACF1 in embryo development, skin integrity maintenance, neural development, bone formation, and colonic paracellular permeability. Mutation in the human MACF1 gene causes a novel myopathy genetic disease. In addition, abnormal expression of MACF1 is associated with schizophrenia, Parkinson's disease, cancer and osteoporosis. This demonstrates the crucial roles of MACF1 in physiology and pathology. Here, we review the research advances of MACF1's roles in specific tissue and in human diseases, providing the perspectives of MACF1 for future studies.Entities:
Keywords: Human disease; Knockout mouse model; MACF1; Spectraplakin; Tissue-specific function
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Year: 2017 PMID: 28577926 DOI: 10.1016/j.semcdb.2017.05.017
Source DB: PubMed Journal: Semin Cell Dev Biol ISSN: 1084-9521 Impact factor: 7.727