Published in J Clin Invest on February 01, 2002
Insulin gene mutations as a cause of permanent neonatal diabetes. Proc Natl Acad Sci U S A (2007) 3.77
IRE1α induces thioredoxin-interacting protein to activate the NLRP3 inflammasome and promote programmed cell death under irremediable ER stress. Cell Metab (2012) 2.80
Neonatal diabetes mellitus. Endocr Rev (2008) 2.64
Seven mutations in the human insulin gene linked to permanent neonatal/infancy-onset diabetes mellitus. J Clin Invest (2008) 2.36
ER stress-mediated autophagy promotes Myc-dependent transformation and tumor growth. J Clin Invest (2012) 1.85
Proinsulin maturation, misfolding, and proteotoxicity. Proc Natl Acad Sci U S A (2007) 1.69
Type 1 diabetic akita mouse hearts are insulin sensitive but manifest structurally abnormal mitochondria that remain coupled despite increased uncoupling protein 3. Diabetes (2008) 1.55
Humanized mouse models to study human diseases. Curr Opin Endocrinol Diabetes Obes (2010) 1.48
Proinsulin disulfide maturation and misfolding in the endoplasmic reticulum. J Biol Chem (2005) 1.36
ER stress and its functional link to mitochondria: role in cell survival and death. Cold Spring Harb Perspect Biol (2011) 1.33
Endoplasmic reticulum stress, pancreatic β-cell degeneration, and diabetes. Cold Spring Harb Perspect Med (2012) 1.23
Apoptosis-inducing signal sequence mutation in carbonic anhydrase IV identified in patients with the RP17 form of retinitis pigmentosa. Proc Natl Acad Sci U S A (2004) 1.20
Genomewide analysis reveals novel pathways affecting endoplasmic reticulum homeostasis, protein modification and quality control. Genetics (2009) 1.15
Proinsulin and the genetics of diabetes mellitus. J Biol Chem (2009) 1.12
Humanized mice for the study of type 1 diabetes and beta cell function. Ann N Y Acad Sci (2008) 1.11
Endoplasmic reticulum (ER) chaperone regulation and survival of cells compensating for deficiency in the ER stress response kinase, PERK. J Biol Chem (2008) 1.11
Chiral mutagenesis of insulin. Foldability and function are inversely regulated by a stereospecific switch in the B chain. Biochemistry (2005) 1.06
Spontaneous development of endoplasmic reticulum stress that can lead to diabetes mellitus is associated with higher calcium-independent phospholipase A2 expression: a role for regulation by SREBP-1. J Biol Chem (2009) 1.05
Diabetes mellitus due to the toxic misfolding of proinsulin variants. FEBS Lett (2013) 1.02
Neuritic dystrophy and neuronopathy in Akita (Ins2(Akita)) diabetic mouse sympathetic ganglia. Exp Neurol (2008) 1.02
Potential for therapeutic manipulation of the UPR in disease. Semin Immunopathol (2013) 0.99
Pro370Leu mutant myocilin disturbs the endoplasm reticulum stress response and mitochondrial membrane potential in human trabecular meshwork cells. Mol Vis (2007) 0.96
The unfolded protein response and autophagy: herpesviruses rule! J Virol (2008) 0.94
Limitation of individual folding resources in the ER leads to outcomes distinct from the unfolded protein response. J Cell Sci (2012) 0.93
Chronic Akt activation attenuated lipopolysaccharide-induced cardiac dysfunction via Akt/GSK3β-dependent inhibition of apoptosis and ER stress. Biochim Biophys Acta (2013) 0.93
Activating transcription factor 6 (ATF6) sequence polymorphisms in type 2 diabetes and pre-diabetic traits. Diabetes (2007) 0.93
Advancing animal models of human type 1 diabetes by engraftment of functional human tissues in immunodeficient mice. Cold Spring Harb Perspect Med (2012) 0.91
Effect of insulin and an erythropoietin-derived peptide (ARA290) on established neuritic dystrophy and neuronopathy in Akita (Ins2 Akita) diabetic mouse sympathetic ganglia. Exp Neurol (2011) 0.90
β cell ER stress and the implications for immunogenicity in type 1 diabetes. Front Cell Dev Biol (2015) 0.90
Diabetic modifier QTLs identified in F2 intercrosses between Akita and A/J mice. Mamm Genome (2006) 0.89
The unfolded protein response in immunity and inflammation. Nat Rev Immunol (2016) 0.86
INS-gene mutations: from genetics and beta cell biology to clinical disease. Mol Aspects Med (2014) 0.86
A new immunodeficient hyperglycaemic mouse model based on the Ins2Akita mutation for analyses of human islet and beta stem and progenitor cell function. Diabetologia (2008) 0.86
The role of endoplasmic reticulum stress in autoimmune-mediated beta-cell destruction in type 1 diabetes. Exp Diabetes Res (2012) 0.85
Contribution of residue B5 to the folding and function of insulin and IGF-I: constraints and fine-tuning in the evolution of a protein family. J Biol Chem (2009) 0.85
Endoplasmic reticulum overcrowding as a mechanism of beta-cell dysfunction in diabetes. Biophys J (2010) 0.84
HLA-B27 misfolding and spondyloarthropathies. Prion (2009) 0.84
Crystal structure of a "nonfoldable" insulin: impaired folding efficiency despite native activity. J Biol Chem (2009) 0.83
Human allograft rejection in humanized mice: a historical perspective. Cell Mol Immunol (2012) 0.83
The role of the unfolded protein response in diabetes mellitus. Semin Immunopathol (2013) 0.81
Insulin: a small protein with a long journey. Protein Cell (2010) 0.81
A general strategy for the bacterial expression of amyloidogenic peptides using BCL-XL-1/2 fusions. Protein Sci (2009) 0.81
Use of Humanized Mice to Study the Pathogenesis of Autoimmune and Inflammatory Diseases. Inflamm Bowel Dis (2015) 0.80
Using Drosophila to discover mechanisms underlying type 2 diabetes. Dis Model Mech (2016) 0.80
The structure and function of insulin: decoding the TR transition. Vitam Horm (2009) 0.79
Genetic modulation of diabetic nephropathy among mouse strains with Ins2 Akita mutation. Physiol Rep (2014) 0.79
Deciphering the hidden informational content of protein sequences: foldability of proinsulin hinges on a flexible arm that is dispensable in the mature hormone. J Biol Chem (2010) 0.79
Inherent ER stress in pancreatic islet β cells causes self-recognition by autoreactive T cells in type 1 diabetes. J Autoimmun (2016) 0.78
Protective mechanisms of mitochondria and heart function in diabetes. Antioxid Redox Signal (2015) 0.78
Causes and consequences of endoplasmic reticulum stress in rheumatic disease. Nat Rev Rheumatol (2016) 0.77
Megsin gene: its genomic analysis, pathobiological functions, and therapeutic perspectives. Curr Genomics (2007) 0.77
β-Cell dysfunction under hyperglycemic stress: a molecular model. J Diabetes Sci Technol (2010) 0.77
Rodent animal models: from mild to advanced stages of diabetic nephropathy. Inflammopharmacology (2014) 0.77
Deciphering a molecular mechanism of neonatal diabetes mellitus by the chemical synthesis of a protein diastereomer, [D-AlaB8]human proinsulin. J Biol Chem (2014) 0.76
A Bystander Mechanism Explains the Specific Phenotype of a Broadly Expressed Misfolded Protein. PLoS Genet (2016) 0.76
Soluble epoxide hydrolase inhibition alleviates neuropathy in Akita (Ins2 Akita) mice. Behav Brain Res (2017) 0.75
Transgenic zebrafish model of the C43G human insulin gene mutation. J Diabetes Investig (2012) 0.75
Environmental Factors Contribute to β Cell Endoplasmic Reticulum Stress and Neo-Antigen Formation in Type 1 Diabetes. Front Endocrinol (Lausanne) (2017) 0.75
Regulated translation initiation controls stress-induced gene expression in mammalian cells. Mol Cell (2000) 17.44
Coupling of stress in the ER to activation of JNK protein kinases by transmembrane protein kinase IRE1. Science (2000) 13.62
Caspase-12 mediates endoplasmic-reticulum-specific apoptosis and cytotoxicity by amyloid-beta. Nature (2000) 13.45
Stress signaling from the lumen of the endoplasmic reticulum: coordination of gene transcriptional and translational controls. Genes Dev (1999) 13.24
CHOP is implicated in programmed cell death in response to impaired function of the endoplasmic reticulum. Genes Dev (1998) 11.86
Dynamic interaction of BiP and ER stress transducers in the unfolded-protein response. Nat Cell Biol (2000) 11.62
Translational control is required for the unfolded protein response and in vivo glucose homeostasis. Mol Cell (2001) 11.21
Perk is essential for translational regulation and cell survival during the unfolded protein response. Mol Cell (2000) 11.09
Diabetes mellitus and exocrine pancreatic dysfunction in perk-/- mice reveals a role for translational control in secretory cell survival. Mol Cell (2001) 8.60
Gadd153 sensitizes cells to endoplasmic reticulum stress by down-regulating Bcl2 and perturbing the cellular redox state. Mol Cell Biol (2001) 8.03
Targeted disruption of the Chop gene delays endoplasmic reticulum stress-mediated diabetes. J Clin Invest (2002) 6.48
Setting the standards: quality control in the secretory pathway. Science (1999) 6.41
Cloning of mammalian Ire1 reveals diversity in the ER stress responses. EMBO J (1998) 5.96
Tripartite management of unfolded proteins in the endoplasmic reticulum. Cell (2000) 5.56
Conformational disease. Lancet (1997) 4.53
Heat shock, stress proteins, chaperones, and proteotoxicity. Cell (1991) 3.96
A mutation in the insulin 2 gene induces diabetes with severe pancreatic beta-cell dysfunction in the Mody mouse. J Clin Invest (1999) 3.86
Ligand-independent dimerization activates the stress response kinases IRE1 and PERK in the lumen of the endoplasmic reticulum. J Biol Chem (2000) 3.40
Procollagen folding and assembly: the role of endoplasmic reticulum enzymes and molecular chaperones. Semin Cell Dev Biol (1999) 2.01
Familial dementia caused by polymerization of mutant neuroserpin. Nature (1999) 1.88
Compensatory responses in mice carrying a null mutation for Ins1 or Ins2. Diabetes (2001) 1.72
Alteration of islet cell populations in spontaneously diabetic mice. Diabetes (1978) 1.60
Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease. J Cell Biol (1998) 1.53
A cellular mechanism governing the severity of Pelizaeus-Merzbacher disease. Nat Genet (1996) 1.39
Overloaded endoplasmic reticulum-Golgi compartments, a possible pathomechanism of peripheral neuropathies caused by mutations of the peripheral myelin protein PMP22. J Neurosci (1998) 1.06
Molecular basis of autosomal dominant neurohypophyseal diabetes insipidus. Cellular toxicity caused by the accumulation of mutant vasopressin precursors within the endoplasmic reticulum. J Clin Invest (1997) 0.95
Alpha-1-antitrypsin deficiency. Semin Liver Dis (1998) 0.94
Protein synthesis. The perks of balancing glucose. Science (2001) 0.91
Tetraspan myelin protein PMP22 and demyelinating peripheral neuropathies: new facts and hypotheses. Glia (2000) 0.83