Intermediate filament interactions can be altered by HSP27 and αB-crystallin
MD Perng, L Cairns, P Van Den IJssel… - Journal of cell …, 1999 - journals.biologists.com
HSP27 and αB-crystallin are both members of the small heat shock protein family. αB-crystalllin
has been proposed to modulate intermediate filaments and recently a mutation in αB-…
has been proposed to modulate intermediate filaments and recently a mutation in αB-…
Comparison of the small heat shock proteins αB-crystallin, MKBP, HSP25, HSP20, and cvHSP in heart and skeletal muscle
N Golenhofen, MD Perng, RA Quinlan… - Histochemistry and cell …, 2000 - Springer
Seven members of the small heat shock protein (sHSP) family are exceptional with respect
to their constitutive high abundance in muscle tissue. It has been suggested that sHSPs …
to their constitutive high abundance in muscle tissue. It has been suggested that sHSPs …
Insights into the beaded filament of the eye lens
MD Perng, Q Zhang, RA Quinlan - Experimental cell research, 2007 - Elsevier
Filensin (BFSP1) and CP49 (BFSP2) represent two members of the IF protein superfamily
that are thus far exclusively expressed in the eye lens. Mutations in both proteins cause lens …
that are thus far exclusively expressed in the eye lens. Mutations in both proteins cause lens …
The cardiomyopathy and lens cataract mutation in alphaB-crystallin alters its protein structure, chaperone activity, and interaction with intermediate filaments in vitro.
MD Perng, PJ Muchowski, P van Den IJssel… - The Journal of …, 1999 - europepmc.org
Desmin-related myopathy and cataract are both caused by the R120G mutation in alphaB-crystallin.
Desmin-related myopathy is one of several diseases characterized by the …
Desmin-related myopathy is one of several diseases characterized by the …
Glial fibrillary acidic protein filaments can tolerate the incorporation of assembly-compromised GFAP-δ, but with consequences for filament organization and αB …
MD Perng, SF Wen, T Gibbon… - Molecular Biology of …, 2008 - Am Soc Cell Biol
The glial fibrillary acidic protein (GFAP) gene is alternatively spliced to give GFAP-α, the
most abundant isoform, and seven other differentially expressed transcripts including GFAP-δ. …
most abundant isoform, and seven other differentially expressed transcripts including GFAP-δ. …
[HTML][HTML] Oligomers of mutant glial fibrillary acidic protein (GFAP) inhibit the proteasome system in Alexander disease astrocytes, and the small heat shock protein αB …
The accumulation of the intermediate filament protein, glial fibrillary acidic protein (GFAP), in
astrocytes of Alexander disease (AxD) impairs proteasome function in astrocytes. We have …
astrocytes of Alexander disease (AxD) impairs proteasome function in astrocytes. We have …
Site-specific phosphorylation and caspase cleavage of GFAP are new markers of Alexander disease severity
…, RA Newman, RA Quinlan, JE Goldman, MD Perng… - Elife, 2019 - elifesciences.org
Alexander disease (AxD) is a fatal neurodegenerative disorder caused by mutations in glial
fibrillary acidic protein (GFAP), which supports the structural integrity of astrocytes. Over 70 …
fibrillary acidic protein (GFAP), which supports the structural integrity of astrocytes. Over 70 …
Caspase cleavage of GFAP produces an assembly-compromised proteolytic fragment that promotes filament aggregation
…, RA Quinlan, A Messing, MD Perng - ASN …, 2013 - journals.sagepub.com
IF (intermediate filament) proteins can be cleaved by caspases to generate proapoptotic
fragments as shown for desmin. These fragments can also cause filament aggregation. The …
fragments as shown for desmin. These fragments can also cause filament aggregation. The …
Antisense therapy in a rat model of Alexander disease reverses GFAP pathology, white matter deficits, and motor impairment
Alexander disease (AxD) is a devastating leukodystrophy caused by gain-of-function
mutations in GFAP, and the only available treatments are supportive. Recent advances in …
mutations in GFAP, and the only available treatments are supportive. Recent advances in …
Alexander disease causing mutations in the C-terminal domain of GFAP are deleterious both to assembly and network formation with the potential to both activate …
…, SC Lim, MH Chen, RA Quinlan, MD Perng - Experimental cell …, 2011 - Elsevier
Alexander disease is a primary genetic disorder of astrocyte caused by dominant mutations
in the astrocyte-specific intermediate filament glial fibrillary acidic protein (GFAP). While most …
in the astrocyte-specific intermediate filament glial fibrillary acidic protein (GFAP). While most …