Abnormal mitochondrial transport and morphology as early pathological changes in human models of spinal muscular atrophy

DMM Disease Models and Mechanisms

Volumn 9, Issue 1, 2016, Pages 39-49

  Xu, Chong Chong ^a   Denton, Kyle R ^a   Wang, Zhi Bo ^a   Zhang, Xiaoqing ^b   Li, Xue Jun ^a,c  

^a UNIVERSITY OF CONNECTICUT HEALTH CENTER   (United States)

^b TONGJI UNIVERSITY SCHOOL OF MEDICINE   (China)

^c UNIVERSITY OF CONNECTICUT   (United States)

Author keywords

HESCs; Human embryonic stem cells; Induced pluripotent stem cells; IPSCs; Mitochondrial transport and morphology; Spinal muscular atrophy

Indexed keywords

ACETYLCYSTEINE; SURVIVAL MOTOR NEURON PROTEIN 1; CASPASE 3; CASPASE 7;

APOPTOSIS; ARTICLE; FOREBRAIN; HUMAN; HUMAN CELL; HUMAN EMBRYONIC STEM CELL; HUMAN EXPERIMENT; MITOCHONDRIAL DYNAMICS; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRION; NERVE CELL CULTURE; NERVE CELL DEGENERATION; NERVE CELL DIFFERENTIATION; NERVE FIBER TRANSPORT; PLURIPOTENT STEM CELL; PRIORITY JOURNAL; PROTEIN EXPRESSION; SPINAL CORD MOTONEURON; SPINAL MUSCULAR ATROPHY; ANIMAL; CELL CULTURE; CELL DIFFERENTIATION; CHEMISTRY; CYTOLOGY; DISEASE MODEL; INDUCED PLURIPOTENT STEM CELL; KARYOTYPING; MEMBRANE POTENTIAL; METABOLISM; MOTONEURON; MOUSE; PATHOLOGY; PATHOPHYSIOLOGY; POLYMERASE CHAIN REACTION; SCID MOUSE; TRANSPORT AT THE CELLULAR LEVEL;

ACETYLCYSTEINE; ANIMALS; BIOLOGICAL TRANSPORT; CASPASE 3; CASPASE 7; CELL DIFFERENTIATION; CELLS, CULTURED; DISEASE MODELS, ANIMAL; HUMANS; INDUCED PLURIPOTENT STEM CELLS; KARYOTYPING; MEMBRANE POTENTIALS; MICE; MICE, SCID; MITOCHONDRIA; MOTOR NEURONS; MUSCULAR ATROPHY, SPINAL; POLYMERASE CHAIN REACTION; PROSENCEPHALON;

EID: 84954470130     PISSN: 17548403     EISSN: 17548411     Source Type: Journal    
DOI: 10.1242/dmm.021766     Document Type: Article

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