Global ETD Search
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Showing 1 to 3 of 3 for “"Gigaxonin"”.
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Role of Gigaxonin in the Regulation of Intermediate Filaments: a Study Using Giant Axonal Neuropathy Patient-Derived Induced Pluripotent Stem Cell-Motor Neurons
… recessive mutations leading to low levels of gigaxonin, a ubiquitously-expressed cytoplasmic protein whose cellular roles are poorly understood. GAN pathology is characterized by aggregates of intermediate filaments (IFs) in multiple tissues. Disorganization of the neuronal intermediate …
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Insights into BTB-Cul3 Ubiquitin Ligases from the Structures of SPOP-Substrate Complexes
… E3s. 3-box is also found in the BTB protein Gigaxonin and is predicted in a subset of BTB proteins. Furthermore, structures of nearly full-length SPOP constructs indicate flexibility between the MATH and BTB domains, potentially allowing regulation of diverse substrates. Those such as Puc …
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Identification of altered Ras signaling and intermediate filament hyperphosphorylation in giant axonal neuropathy
… GAN is caused by reduced levels of the gigaxonin (Giga) protein. Giga functions to control the degradation of other cellular proteins, and the loss of Giga in GAN cells results in significantly elevated levels of the galectin-1 (Gal-1) protein. Gal-1 stabilizes the active form of the Ras …