Zobrazeno 1 - 10
of 102
pro vyhledávání: '"Denis I. Crane"'
Autor:
Yongjun Fan, Gautam Wali, Ratneswary Sutharsan, Bernadette Bellette, Denis I. Crane, Carolyn M. Sue, Alan Mackay-Sim
Publikováno v:
Biology Open, Vol 3, Iss 6, Pp 494-502 (2014)
Hereditary Spastic Paraplegia (HSP) is a genetically heterogeneous group of disorders, diagnosed by progressive gait disturbances with muscle weakness and spasticity, for which there are no treatments targeted at the underlying pathophysiology. Mutat
Externí odkaz:
https://doaj.org/article/0e25420270d44897989da33cb4a75dfe
Autor:
C. Catharina Müller, Tam H. Nguyen, Barbara Ahlemeyer, Mallika Meshram, Nishreen Santrampurwala, Siyu Cao, Peter Sharp, Pamela B. Fietz, Eveline Baumgart-Vogt, Denis I. Crane
Publikováno v:
Disease Models & Mechanisms, Vol 4, Iss 1, Pp 104-119 (2011)
SUMMARY Delayed cerebellar development is a hallmark of Zellweger syndrome (ZS), a severe neonatal neurodegenerative disorder. ZS is caused by mutations in PEX genes, such as PEX13, which encodes a protein required for import of proteins into the per
Externí odkaz:
https://doaj.org/article/5633aacc1f4d4ca1a76cfcc0e8cca5d4
Publikováno v:
The Scientific World Journal, Vol 2013 (2013)
Earlier findings from our laboratory implicated RhoA in heart developmental processes. To investigate factors that potentially regulate RhoA expression, RhoA gene organisation and promoter activity were analysed. Comparative analysis indicated strict
Externí odkaz:
https://doaj.org/article/2256c55771594abeb4ee03a2364f433b
Autor:
Eriza S. Secondes, Denis I. Crane, Michelle Melino, Maneet Bhatia, Gautam Rishi, V. Nathan Subramaniam
Publikováno v:
Biochimica et biophysica acta. Molecular basis of disease. 1866(10)
Peroxisomes are organelles, abundant in the liver, involved in a variety of cellular functions, including fatty acid metabolism, plasmalogen synthesis and metabolism of reactive oxygen species. Several inherited disorders are associated with peroxiso
Publikováno v:
Disaster Prevention and Management: An International Journal. 27:447-455
Purpose The purpose of this paper is to reveal difficulties associated with identifying child victims of the 2004 South-East Asia Tsunami at the Thai Tsunami Victim Identification (TTVI) operation in Phuket and explores two strategies that increased
Autor:
Prashant Mishra, Shyam Sirasanagandla, Rhea Sumpter, Yongjie Wei, Beth Levine, Denis I. Crane, Ming Y Lee, Zhongju Zou, Hendrik Rosewich
Publikováno v:
EMBO Reports
PEX13 is an integral membrane protein on the peroxisome that regulates peroxisomal matrix protein import during peroxisome biogenesis. Mutations in PEX13 and other peroxin proteins are associated with Zellweger syndrome spectrum (ZSS) disorders, a su
Publikováno v:
Neuroscience. 334:201-213
Zellweger syndrome (ZS) is a peroxisome biogenesis disorder that involves significant neuropathology, the molecular basis of which is still poorly understood. Using a mouse model of ZS with brain-restricted deficiency of the peroxisome biogenesis pro
Publikováno v:
Forensic Science Policy & Management: An International Journal. 6:69-78
The Thai Tsunami Victim Identification operation was the largest disaster victim identification operation in history, involving 3,679 unidentified victims from 41 countries, and police and forensic scientists from over 30 countries. This paper evalua
Publikováno v:
Molecular and cellular neurosciences. 88
Zellweger syndrome (ZS), a neonatal lethal disorder arising from defective peroxisome biogenesis, features profound neuroanatomical abnormalities and brain dysfunction. Here we used mice with brain-restricted inactivation of the peroxisome biogenesis
Publikováno v:
Neuroscience. 274:229-241
Zellweger syndrome (ZS) is a severe peroxisomal disorder caused by mutations in peroxisome biogenesis, or PEX, genes. A central hallmark of ZS is abnormal neuronal migration and neurodegeneration, which manifests as widespread neurological dysfunctio