Zobrazeno 1 - 10
of 117
pro vyhledávání: '"Jinwoong Bok"'
Autor:
Song Yi Youn, Hyehyun Min, Se Rok Jeong, Jiahn Lee, Seok Jun Moon, Jinwoong Bok, Chul Hoon Kim
Publikováno v:
Molecular Brain, Vol 15, Iss 1, Pp 1-4 (2022)
Abstract Tubby mice exhibit hearing impairment due to the loss of stereocilin from the tip regions that connect the tallest stereocilia of the outer hair cells (OHCs) to the tectorial membrane. Stereocilin is an essential stereociliary protein in the
Externí odkaz:
https://doaj.org/article/096e47d2689244739886f42578135b84
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 11 (2023)
The cochlea is a complex organ comprising diverse cell types with highly specialized morphology and function. Until now, the molecular underpinnings of its specializations have mostly been studied from a transcriptional perspective, but accumulating
Externí odkaz:
https://doaj.org/article/60e4da68e6ee4af6ac9293dca6928162
Publikováno v:
Frontiers in Cell and Developmental Biology, Vol 10 (2022)
Primary cilia have essential roles as signaling centers during development and adult homeostasis. Disruption of ciliary structure or function causes congenital human disorders called ciliopathies. Centriolar distal appendage (DAP) proteins are import
Externí odkaz:
https://doaj.org/article/318ca573bd304b89ad05d928ac75d72e
Autor:
Ji-Hyun Ma, Eunju Lee, Sung-Hyun Yoon, Hyehyun Min, Jae Hwan Oh, Inhwa Hwang, Yejin Sung, Ju Hee Ryu, Jinwoong Bok, Je-Wook Yu
Publikováno v:
EBioMedicine, Vol 82, Iss , Pp 104184- (2022)
Summary: Background: Cryopyrin-associated periodic syndrome (CAPS) is an inherited autoinflammatory disease caused by a gain-of-function mutation in NLRP3. Although CAPS patients frequently suffer from sensorineural hearing loss, it remains unclear w
Externí odkaz:
https://doaj.org/article/2df006bd2eca4d339110b2bb1456596a
Publikováno v:
Frontiers in Molecular Biosciences, Vol 8 (2021)
Alternative splicing (AS) refers to the production of multiple mRNA isoforms from a single gene due to alternative selection of exons or splice sites during pre-mRNA splicing. It is a primary mechanism of gene regulation in higher eukaryotes and sign
Externí odkaz:
https://doaj.org/article/61e489948dd14fd4ac0d83494075c6ea
Publikováno v:
EBioMedicine, Vol 49, Iss , Pp 305-317 (2019)
Background: Endocrine-cerebro-osteodysplasia (ECO) syndrome is a genetic disorder associated with congenital defects of the endocrine, cerebral, and skeletal systems in humans. ECO syndrome is caused by mutations of the intestinal cell kinase (ICK) g
Externí odkaz:
https://doaj.org/article/ce90877efd73406c872860a8c5b772a3
Autor:
Min-A Kim, Nari Ryu, Hye-Min Kim, Ye-Ri Kim, Byeonghyeon Lee, Tae-Jun Kwon, Jinwoong Bok, Un-Kyung Kim
Publikováno v:
Molecular Therapy: Methods & Clinical Development, Vol 13, Iss , Pp 197-204 (2019)
Targeting specific cell types in the mammalian inner ear is important for treating genetic hearing loss due to the different cell type-specific functions. Adeno-associated virus (AAV) is an efficient in vivo gene transfer vector, and it has demonstra
Externí odkaz:
https://doaj.org/article/b7c1714cb9374824b6a5cf08af193951
Autor:
Ye-Ri Kim, Jeong-In Baek, Sung Hwan Kim, Min-A Kim, Byeonghyeon Lee, Nari Ryu, Kyung-Hee Kim, Deok-Gyun Choi, Hye-Min Kim, Michael P. Murphy, Greg Macpherson, Yeon-Sik Choo, Jinwoong Bok, Kyu-Yup Lee, Jeen-Woo Park, Un-Kyung Kim
Publikováno v:
Redox Biology, Vol 20, Iss , Pp 544-555 (2019)
Mitochondrial NADP+-dependent isocitrate dehydrogenase 2 (IDH2) is a major NADPH-producing enzyme which is essential for maintaining the mitochondrial redox balance in cells. We sought to determine whether IDH2 deficiency induces mitochondrial dysfun
Externí odkaz:
https://doaj.org/article/d71b374266564676820b6c5f775c1afa
Autor:
Kyeong-Hye Moon, Ji-Hyun Ma, Hyehyun Min, Heiyeun Koo, HongKyung Kim, Hyuk Wan Ko, Jinwoong Bok
Publikováno v:
eLife, Vol 9 (2020)
Defective primary cilia cause a range of diseases known as ciliopathies, including hearing loss. The etiology of hearing loss in ciliopathies, however, remains unclear. We analyzed cochleae from three ciliopathy mouse models exhibiting different cili
Externí odkaz:
https://doaj.org/article/f72a4072fec3427fa028badeca639eea
Autor:
Bo Gyung Kim, Jin Young Kim, Hee Nam Kim, Jinwoong Bok, Wan Namkung, Jae Young Choi, Sung Huhn Kim
Publikováno v:
PLoS ONE, Vol 9, Iss 4, p e95730 (2014)
Pendrin mutations cause enlarged vestibular aqueducts and various degrees of sensorineural hearing loss. The selective abolition of pendrin causes dilation of the membranous labyrinth known as endolymphatic hydrops, loss of the endocochlear potential
Externí odkaz:
https://doaj.org/article/bfb0235aa25244738b0dccaf961e1fad