Selected Publications

Academic Article

Year Title Altmetric
2022 Corrigendum to: Type II Alexander disease caused by splicing errors and aberrant overexpression of an uncharacterized GFAP isoform (Human Mutation, (2020), 41, 6, (1131-1137), 10.1002/humu.24008)Human Mutation.  43:1344. 2022
2021 Regulation of GFAP ExpressionASN Neuro.  13. 2021
2020 Type II Alexander disease caused by splicing errors and aberrant overexpression of an uncharacterized GFAP isoformHuman Mutation.  41:1131-1137. 2020
2020 GFAP at 50ASN Neuro.  12. 2020
2019 Relative stabilities of wild-type and mutant glial fibrillary acidic protein in patients with Alexander diseaseJournal of Biological Chemistry.  294:15604-15612. 2019
2019 AP-1 and the injury response of the GFAP geneJournal of Neuroscience Research.  97:149-161. 2019
2016 Composition of Rosenthal Fibers, the Protein Aggregate Hallmark of Alexander DiseaseJournal of Proteome Research.  15:2265-2282. 2016
2015 Elevated GFAP induces astrocyte dysfunction in caudal brain regions: A potential mechanism for hindbrain involved symptoms in type II Alexander diseaseGlia.  63:2285-2297. 2015
2015 A new mutation in GFAP widens the spectrum of Alexander diseaseEuropean Journal of Human Genetics.  23:1-2. 2015
2014 Changes in the morphology and proliferation of astrocytes induced by two modalities of chemically functionalized single-walled carbon nanotubes are differentially mediated by glial fibrillary acidic proteinNano Letters: a journal dedicated to nanoscience and nanotechnology.  14:3720-3727. 2014
2014 Role of GFAP in CNS injuriesNeuroscience Letters.  565:7-13. 2014
2013 Transgenic analysis of GFAP promoter elementsGlia.  61:1488-1499. 2013
2012 Splice site, frameshift, and chimeric GFAP mutations in Alexander diseaseHuman Mutation.  33:1141-1148. 2012
2012 Archetypal and new families with Alexander disease and novel mutations in GFAPArchives of Neurology.  69:208-214. 2012
2011 GFAP mutations, age at onset, and clinical subtypes in Alexander diseaseNeurology.  77:1287-1294. 2011
2010 Alteration Of glial-neuronal metabolic interactions in a mouse model of Alexander diseaseGlia.  58:1228-1234. 2010
2010 Drug screening to identify suppressors of GFAP expressionHuman Molecular Genetics.  19:3169-3178. 2010
2009 An infantile case of Alexander disease unusual for its MRI features and a GFAP allele carrying both the p.Arg79His mutation and the p.Glu223Gln coding variantJournal of Neurology.  256:679-682. 2009
2008 Enhancement of cell-specific transgene expression from a Tet-Off regulatory system using a transcriptional amplification strategy in the rat brainJournal of Gene Medicine.  10:583-592. 2008
2008 GFAP promoter elements required for region-specific and astrocyte-specific expressionGlia.  56:481-493. 2008
2008 Adult-onset Alexander disease with progressive ataxia and palatal tremorMovement Disorders.  23:118-122. 2008
2007 GFAP and its role in Alexander diseaseExperimental Cell Research.  313:2077-2087. 2007
2006 Enhanced hippocampal neurodegeneration after traumatic or kainate excitotoxicity in GFAP-null miceJournal of Clinical Neuroscience.  13:934-938. 2006
2006 Astrocyte heterogeneity revealed by expression of a GFAP-LacZ transgeneGlia.  53:677-687. 2006
2006 Increased glia-specific transgene expression with glial fibrillary acidic protein promoters containing multiple enhancer elementsJournal of Neuroscience Research.  83:744-753. 2006
2006 Propensity for paternal inheritance of de novo mutations in Alexander diseaseHuman Genetics.  119:137-144. 2006
2006 The Alexander disease-causing glial fibrillary acidic protein mutant, R416W, accumulates into Rosenthal fibers by a pathway that involves filament aggregation and the association of αB-crystallin and HSP27American Journal of Human Genetics.  79:197-213. 2006
2005 Erratum: Unusual variants of Alexander's disease (Annals of Neurology (2005) 57 (327-338))Annals of Neurology.  58:172. 2005
2005 Alexander-disease mutation of GFAP causes filament disorganization and decreased solubility of GFAPJournal of Cell Science.  118:2057-2065. 2005
2005 Glial fibrillary acidic protein mutations in infantile, juvenile, and adult forms of Alexander diseaseAnnals of Neurology.  57:310-326. 2005
2005 Unusual variants of Alexander's diseaseAnnals of Neurology.  57:327-338. 2005
2004 Expression specificity of GFAP transgenesNeurochemical Research.  29:2075-2093. 2004
2004 Expression specificity of GFAP transgenes.Neurochemical Research.  29:2075-2093. 2004
2004 Dominantly-inherited adult-onset leukodystrophy with palatal tremor caused by a mutation in the glial fibrillary acidic protein geneMovement Disorders.  19:1244-1248. 2004
2004 Expression of glial fibrillary acidic protein in primary cultures of human Müller cellsExperimental Eye Research.  79:423-429. 2004
2003 Alexander's disease: Clinical, pathologic, and genetic featuresJournal of Child Neurology.  18:625-632. 2003
2003 GFAP: Functional implications gleaned from studies of genetically engineered miceGlia.  43:87-90. 2003
2003 Alexander disease: GFAP mutations unify young and oldLancet Neurology.  2:75. 2003
2002 GFAP mutations in Alexander diseaseInternational Journal of Developmental Neuroscience.  20:259-268. 2002
2002 Molecular findings in symptomatic and pre-symptomatic Alexander disease patientsNeurology.  58:1494-1500. 2002
2002 ICP monitoring following bilateral carotid occlusion in GFAP-null miceActa Neurochirurgica: Supplementum.  81:269-270. 2002
2001 hGFAP-cre transgenic mice for manipulation of glial and neuronal function in vivoGenesis: The Journal of Genetics and Development.  31:85-94. 2001
2001 A novel mutation in glial fibrillary acidic protein gene in a patient with Alexander diseaseNeuroscience Letters.  312:71-74. 2001
2001 Differential cellular expression of tumor necrosis factor-α and Type I tumor necrosis factor receptor after transient global forebrain ischemiaJournal of the Neurological Sciences.  186:87-99. 2001
2001 Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander diseaseNature Genetics.  27:117-120. 2001
2001 Alexander disease: New insights from geneticsJournal of Neuropathology and Experimental Neurology.  60:563-573. 2001
2001 Infantile Alexander disease: Spectrum of GFAP mutations and genotype-phenotype correlationAmerican Journal of Human Genetics.  69:1134-1140. 2001
2000 High susceptibility to cerebral ischemia in GFAP-null miceJournal of Cerebral Blood Flow and Metabolism.  20:1040-1044. 2000
2000 Inhibition of glioma cells in vitro and in vivo using a recombinant adenoviral vector containing an astrocyte-specific promoterCancer Gene Therapy.  7:1120-1126. 2000
1999 Gene therapy in a rodent model of Parkinson's disease using differentiated C6 cells expressing a GFAP-tyrosine hydroxylase transgeneLife Sciences.  65:483-491. 1999
1999 Biosynthetic capacity of C6 cells engineered with a glial fibrillary acidic protein-tyrosine hydroxylase transgeneMedical science research.  27:423-425. 1999
1999 Nonlethal detection of transgene expression in the CNS of founder miceBioTechniques: the journal of laboratory technology for bioresearch.  26:302-307. 1999
1999 TNF-α pretreatment prevents subsequent activation of cultured brain cells with TNF-α and hypoxia via ceramideAmerican Journal of Physiology: Cell Physiology.  276. 1999
1998 Selective killing of glioma cell lines using an astrocyte-specific expression of the herpes simplex virus-thymidine kinase geneCancer Research.  58:4577-4580. 1998
1998 Astrocytes cultured from transgenic mice carrying the added human glial fibrillary acidic protein gene contain rosenthal fibersJournal of Neuroscience Research.  53:353-360. 1998
1998 Mice lacking GFAP are hypersensitive to traumatic cerebrospinal injuryNeuroReport.  9:1691-1696. 1998
1998 Differentiation-dependent expression of transgenes in engineered astrocyte cell linesNeuroscience Letters.  242:172-176. 1998
1998 Fatal encephalopathy with astrocyte inclusions in GFAP transgenic miceAmerican Journal of Pathology.  152:391-398. 1998
1998 Astrocyte-specific expression of tyrosine hydroxylase after intracerebral gene transfer induces behavioral recovery in experimental ParkinsonismGene Therapy (Basingstoke).  5:1650-1655. 1998
1998 Chronic relapsing experimental autoimmune encephalomyelitis: Effects of insulin-like growth factor-I treatment on clinical deficits, lesion severity, glial responses, and blood brain barrier defectsJournal of Neuropathology and Experimental Neurology.  57:426-438. 1998
1998 Stimulation of tyrosine phosphorylation of a brain protein by hibernationJournal of Cerebral Blood Flow and Metabolism.  18:1040-1045. 1998
1997 Global forebrain ischemia results in differential cellular expression of interleukin-1β (IL-1β) and its receptor at mRNA and protein levelJournal of Cerebral Blood Flow and Metabolism.  17:1107-1120. 1997
1996 Decreased calcium accumulation in isolated nerve endings during hibernation in ground squirrelsNeurochemical Research.  21:947-954. 1996
1996 GFAP transgenic miceMethods.  10:351-364. 1996
1996 Somatostatin as a trophic factor: Analysis of transgenic mice overexpressing somatostatin in astrocytesAnnals of the New York Academy of Sciences.  780:29-35. 1996
1995 A novel glial fibrillary acidic protein mRNA lacking exon 1Brain Research.  30:251-258. 1995
1995 Astrocytes Upregulate Glial Fibrillary Acidic Protein (GFAP), but not Insulin‐like Growth Factor‐I (IGF‐I) during Experimental Autoimmune Neuritis (EAN)Brain Pathology.  5:1-10. 1995
1995 Cryogenic spinal cord injury induces astrocytic gene expression of insulin‐like growth factor I and insulin‐like growth factor binding protein 2 during myelin regenerationJournal of Neuroscience Research.  40:647-659. 1995
1995 Overexpression of TGF-ß1 in the central nervous system of transgenic mice results in hydrocephalusJournal of Neuropathology and Experimental Neurology.  54:339-349. 1995
1994 Astrocytes express insulin-like growth factor-I (IGF-I) and its binding protein, IGFBP-2, during demyelination induced by experimental autoimmune encephalomyelitisMolecular and Cellular Neuroscience.  5:418-430. 1994
1994 Concentric sclerosis (Baló): Morphometric and in situ hybridization study of lesions in six patientsAnnals of Neurology.  35:18-30. 1994
1994 Cyclic AMP Decreases the Expression of a Neuronal Marker (GAD67) and Increases the Expression of an Astroglial Marker (GFAP) in C6 CellsJournal of Neurochemistry.  63:1218-1225. 1994
1994 Expression of insulin-like growth factor-I and related peptides during motoneuron regenerationExperimental Neurology.  128:202-210. 1994
1994 Structure and Transcriptional Regulation of the GFAP GeneBrain Pathology.  4:245-257. 1994
1993 Analysis of a Segment of the Human Glial Fibrillary Acidic Protein Gene That Directs Astrocyte‐Specific TranscriptionJournal of Neurochemistry.  61:160-166. 1993
1991 Multiple interacting sites regulate astrocyte-specific transcription of the human gene for glial fibrillary acidic proteinJournal of Biological Chemistry.  266:18877-18883. 1991
1990 A downstream initiation element required for efficient TATA box binding and in vitro function of TFIIDNature.  348:86-88. 1990
1990 Characterization of human cDNA and genomic clones for glial fibrillary acidic proteinBrain Research.  7:277-286. 1990
1989 Rom transcript of plasmid ColE1Nucleic Acids Research.  17:4309-4326. 1989
1985 The role of enzyme sequestration in the regulation of the adenylate cyclase of Dictyostelium discoideumJournal of Biological Chemistry.  260:3613-3616. 1985
1983 Forskolin does not activate cyclic AMP synthesis in Dictyostelium discoideum in vivo or in vitroJournal of cyclic nucleotide and protein phosphorylation research.  9:297-303. 1983


Year Title Altmetric
2017 The significance of deiminated GFAP in neurodegenerative diseases with special emphasis on alexander disease.  391-412. 2017
2016 Alexander disease.  23-26. 2016
2014 Alexander disease and astrotherapeutics.  89-105. 2014
2014 Alexander's Disease.  106-109. 2014
2014 Ongoing studies of deimination in neurodegenerative diseases using the F95 antibody.  257-280. 2014
2009 Alexander disease: A genetic disorder of astrocytes.  591-648. 2009
2004 Models of Alexander Disease.  1115-1124. 2004

Education And Training

  • Doctor of Philosophy in Biochemistry, University of California System : Berkeley 1970
  • Full Name

  • Michael Brenner