FRINK Linked Data Fragments server

Matches in SemOpenAlex for { <https://semopenalex.org/work/W1985838757> ?p ?o ?g. }

• W1985838757 endingPage "173" @default.
• W1985838757 startingPage "163" @default.
• W1985838757 abstract "The small heat shock protein HSPB1 is a multifunctional, α-crystallin-based protein that has been shown to be neuroprotective in animal models of motor neuron disease and peripheral nerve injury. Missense mutations in HSPB1 result in axonal Charcot–Marie–Tooth disease with minimal sensory involvement (CMT2F) and distal hereditary motor neuropathy type 2 (dHMN-II). These disorders are characterized by a selective loss of motor axons in peripheral nerve resulting in distal muscle weakness and often severe disability. To investigate the pathogenic mechanisms of HSPB1 mutations in motor neurons in vivo, we have developed and characterized transgenic PrP-HSPB1 and PrP-HSPB1(R136W) mice. These mice express the human HSPB1 protein throughout the nervous system including in axons of peripheral nerve. Although both mouse strains lacked obvious motor deficits, the PrP-HSPB1(R136W) mice developed an age-dependent motor axonopathy. Mutant mice showed axonal pathology in spinal cord and peripheral nerve with evidence of impaired neurofilament cytoskeleton, associated with organelle accumulation. Accompanying these findings, increases in the number of Schmidt–Lanterman incisures, as evidence of impaired axon–Schwann cell interactions, were present. These observations suggest that overexpression of HSPB1(R136W) in neurons is sufficient to cause pathological and electrophysiological changes in mice that are seen in patients with hereditary motor neuropathy." @default.
• W1985838757 created "2016-06-24" @default.
• W1985838757 creator A5006399262 @default.
• W1985838757 creator A5016629548 @default.
• W1985838757 creator A5016640472 @default.
• W1985838757 creator A5040381323 @default.
• W1985838757 creator A5049491454 @default.
• W1985838757 creator A5075758666 @default.
• W1985838757 creator A5079946257 @default.
• W1985838757 creator A5090946680 @default.
• W1985838757 date "2012-08-01" @default.
• W1985838757 modified "2023-10-16" @default.
• W1985838757 title "Mutant HSPB1 overexpression in neurons is sufficient to cause age-related motor neuronopathy in mice" @default.
• W1985838757 cites W1615752294 @default.
• W1985838757 cites W1965589408 @default.
• W1985838757 cites W1972380090 @default.
• W1985838757 cites W1972720074 @default.
• W1985838757 cites W1987220770 @default.
• W1985838757 cites W1991124984 @default.
• W1985838757 cites W1993064034 @default.
• W1985838757 cites W1994700524 @default.
• W1985838757 cites W1997458926 @default.
• W1985838757 cites W2000461947 @default.
• W1985838757 cites W2002307665 @default.
• W1985838757 cites W2003301707 @default.
• W1985838757 cites W2012851706 @default.
• W1985838757 cites W2019313913 @default.
• W1985838757 cites W2020230011 @default.
• W1985838757 cites W2023701085 @default.
• W1985838757 cites W2027014645 @default.
• W1985838757 cites W2032415844 @default.
• W1985838757 cites W2046439284 @default.
• W1985838757 cites W2052915293 @default.
• W1985838757 cites W2064543601 @default.
• W1985838757 cites W2071422853 @default.
• W1985838757 cites W2071668766 @default.
• W1985838757 cites W2073765056 @default.
• W1985838757 cites W2073864172 @default.
• W1985838757 cites W2076812179 @default.
• W1985838757 cites W2077831889 @default.
• W1985838757 cites W2079291061 @default.
• W1985838757 cites W2079870118 @default.
• W1985838757 cites W2086488998 @default.
• W1985838757 cites W2088210081 @default.
• W1985838757 cites W2107660300 @default.
• W1985838757 cites W2110633413 @default.
• W1985838757 cites W2111692215 @default.
• W1985838757 cites W2113559596 @default.
• W1985838757 cites W2115249910 @default.
• W1985838757 cites W2123243347 @default.
• W1985838757 cites W2129621958 @default.
• W1985838757 cites W2142845441 @default.
• W1985838757 cites W2143850427 @default.
• W1985838757 cites W2146003518 @default.
• W1985838757 cites W2149151488 @default.
• W1985838757 cites W2152691363 @default.
• W1985838757 cites W2158265014 @default.
• W1985838757 cites W2159457567 @default.
• W1985838757 cites W2162508192 @default.
• W1985838757 cites W2162901119 @default.
• W1985838757 cites W2407064831 @default.
• W1985838757 doi "https://doi.org/10.1016/j.nbd.2012.03.035" @default.
• W1985838757 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3367118" @default.
• W1985838757 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/22521462" @default.
• W1985838757 hasPublicationYear "2012" @default.
• W1985838757 type Work @default.
• W1985838757 sameAs 1985838757 @default.
• W1985838757 citedByCount "35" @default.
• W1985838757 countsByYear W19858387572012 @default.
• W1985838757 countsByYear W19858387572013 @default.
• W1985838757 countsByYear W19858387572014 @default.
• W1985838757 countsByYear W19858387572015 @default.
• W1985838757 countsByYear W19858387572016 @default.
• W1985838757 countsByYear W19858387572017 @default.
• W1985838757 countsByYear W19858387572018 @default.
• W1985838757 countsByYear W19858387572019 @default.
• W1985838757 countsByYear W19858387572020 @default.
• W1985838757 countsByYear W19858387572021 @default.
• W1985838757 countsByYear W19858387572022 @default.
• W1985838757 crossrefType "journal-article" @default.
• W1985838757 hasAuthorship W1985838757A5006399262 @default.
• W1985838757 hasAuthorship W1985838757A5016629548 @default.
• W1985838757 hasAuthorship W1985838757A5016640472 @default.
• W1985838757 hasAuthorship W1985838757A5040381323 @default.
• W1985838757 hasAuthorship W1985838757A5049491454 @default.
• W1985838757 hasAuthorship W1985838757A5075758666 @default.
• W1985838757 hasAuthorship W1985838757A5079946257 @default.
• W1985838757 hasAuthorship W1985838757A5090946680 @default.
• W1985838757 hasBestOaLocation W19858387572 @default.
• W1985838757 hasConcept C102230213 @default.
• W1985838757 hasConcept C104317684 @default.
• W1985838757 hasConcept C134018914 @default.
• W1985838757 hasConcept C141035611 @default.
• W1985838757 hasConcept C142724271 @default.
• W1985838757 hasConcept C157207234 @default.
• W1985838757 hasConcept C169760540 @default.
• W1985838757 hasConcept C204232928 @default.
• W1985838757 hasConcept C25498285 @default.

• next