Dominant protein interactions that influence the pathogenesis of conformational diseases

Translational attenuation differentially alters the fate of disease-associated fibulin proteins

(2012) John D. Hulleman et al.   FASEB JOURNAL

Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann–Pick type C disease fibroblasts

(2012) Ting Yu et al.   HUMAN MOLECULAR GENETICS

Proinsulin Intermolecular Interactions during Secretory Trafficking in Pancreatic β Cells

(2012) Leena Haataja et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Delicate Balance Between Secreted Protein Folding and Endoplasmic Reticulum-Associated Degradation in Human Physiology

(2012) Christopher J. Guerriero et al.   PHYSIOLOGICAL REVIEWS

A Model of Threshold Behavior Reveals Rescue Mechanisms of Bystander Proteins in Conformational Diseases

(2011) Conner I. Sandefur et al.   BIOPHYSICAL JOURNAL

Lacidipine Remodels Protein Folding and Ca2+ Homeostasis in Gaucher's Disease Fibroblasts: A Mechanism to Rescue Mutant Glucocerebrosidase

(2011) Fan Wang et al.   CHEMISTRY & BIOLOGY

In Vivo Misfolding of Proinsulin Below the Threshold of Frank Diabetes

(2011) I. Hodish et al.   DIABETES

Control of Precursor Maturation and Disposal Is an Early Regulative Mechanism in the Normal Insulin Production of Pancreatic β-Cells

(2011) Jie Wang et al.   PLoS One

Combination therapy utilizing shRNA knockdown and an optimized resistant transgene for rescue of diseases caused by misfolded proteins

(2011) C. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Engineered antibody therapies to counteract mutant huntingtin and related toxic intracellular proteins

(2011) David C. Butler et al.   PROGRESS IN NEUROBIOLOGY

CFTR: folding, misfolding and correcting the ΔF508 conformational defect

(2011) Gergely L. Lukacs et al.   TRENDS IN MOLECULAR MEDICINE

PERK (EIF2AK3) Regulates Proinsulin Trafficking and Quality Control in the Secretory Pathway

(2010) Sounak Gupta et al.   DIABETES

CisandTransActions of the Cholinesterase-like Domain within the Thyroglobulin Dimer

(2010) Xiaofan Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Genetics and phenomics of hypothyroidism and goiter due to thyroglobulin mutations

(2010) Héctor M. Targovnik et al.   MOLECULAR AND CELLULAR ENDOCRINOLOGY

Mutant INS-Gene Induced Diabetes of Youth: Proinsulin Cysteine Residues Impose Dominant-Negative Inhibition on Wild-Type Proinsulin Transport

(2010) Ming Liu et al.   PLoS One

Proinsulin misfolding and diabetes: mutant INS gene-induced diabetes of youth

(2010) Ming Liu et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

In vitro processing and secretion of mutant insulin proteins that cause permanent neonatal diabetes

(2009) Sindhu Rajan et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

The Cholinesterase-like Domain, Essential in Thyroglobulin Trafficking for Thyroid Hormone Synthesis, Is Required for Protein Dimerization

(2009) Jaemin Lee et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Misfolded Proinsulin Affects Bystander Proinsulin in Neonatal Diabetes

(2009) Israel Hodish et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Drug development and the cellular quality control system

(2009) P. Michael Conn et al.   TRENDS IN PHARMACOLOGICAL SCIENCES

The Unfolded Protein Response: A Pathway That Links Insulin Demand with β-Cell Failure and Diabetes

(2008) Donalyn Scheuner et al.   ENDOCRINE REVIEWS

Pharmacological manipulation of gain-of-function and dominant-negative mechanisms in rhodopsin retinitis pigmentosa

(2008) Hugo F. Mendes et al.   HUMAN MOLECULAR GENETICS

The cholinesterase-like domain of thyroglobulin functions as an intramolecular chaperone

(2008) Jaemin Lee et al.   JOURNAL OF CLINICAL INVESTIGATION

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE