The E3 ubiquitin ligase TRIM11 mediates the degradation of congenital central hypoventilation syndrome-associated polyalanine-expanded PHOX2B

In vitro drug treatments reduce the deleterious effects of aggregates containing polyAla expanded PHOX2B proteins

(2011) Eleonora Di Zanni et al.   NEUROBIOLOGY OF DISEASE

An Official ATS Clinical Policy Statement: Congenital Central Hypoventilation Syndrome

(2010) Debra E. Weese-Mayer et al.   AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE

Intranuclear Degradation of Polyglutamine Aggregates by the Ubiquitin-Proteasome System

(2009) Atsushi Iwata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

BAG1 modulates huntingtin toxicity, aggregation, degradation, and subcellular distribution

(2009) Kamila Sroka et al.   JOURNAL OF NEUROCHEMISTRY

Molecular mechanisms underlying polyalanine diseases

(2009) C. Messaed et al.   NEUROBIOLOGY OF DISEASE

Trim11 increases expression of dopamine β-hydroxylase gene by interacting with Phox2b

(2008) Seok Jong Hong et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

PABPN1 polyalanine tract deletion and long expansions modify its aggregation pattern and expression

(2008) Arnaud F. Klein et al.   EXPERIMENTAL CELL RESEARCH

Trim11 modulates the function of neurogenic transcription factor Pax6 through ubiquitin-proteosome system

(2008) T. C. Tuoc et al.   GENES & DEVELOPMENT

E6-AP Promotes Misfolded Polyglutamine Proteins for Proteasomal Degradation and Suppresses Polyglutamine Protein Aggregation and Toxicity

(2008) Amit Mishra et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A human mutation in Phox2b causes lack of CO2 chemosensitivity, fatal central apnea, and specific loss of parafacial neurons

(2008) V. Dubreuil et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA