PedAM
Pediatric Disease Annotations & Medicines
| Disease | maturity-onset diabetes of the young |
| Phenotype | |maturity-onset diabetes of the young |
| Sentences | 137 |
| PubMedID- 23704680 | Among the disease features are the balance between insulin deficiency and insulin insensitivity, fasting versus postprandial hyperglycemia, short versus long disease duration, and special circumstances such as maturity-onset diabetes of the young (mody) or latent autoimmune diabetes in adulthood (lada). |
| PubMedID- 25619391 | Liraglutide is effective in patients with maturity-onset diabetes of the young (mody) 3, and produces lower hypoglycemic risk than glimepiride9. |
| PubMedID- 21294870 | Eight subjects with genetically verified maturity-onset diabetes of the young [24] and 205 subjects evaluated as having type 1 diabetes were excluded. |
| PubMedID- 21951399 | Diabetes caused by a single gene mutation has also been called maturity-onset diabetes of the young (mody) [13,14]. |
| PubMedID- 22110178 | (2), which demonstrated that high-sensitivity c-reactive protein (hs-crp) levels are lower in hepatocyte nuclear factor 1-α (hnf1a) maturity-onset diabetes of the young (mody) patients in comparison with other forms of diabetes, including type 2 diabetes. |
| PubMedID- 22065581 | We screened 18 diabetic probands with unelucidated maturity-onset diabetes of the young (mody). |
| PubMedID- 23251491 | However, only the maturity-onset diabetes of the young (mody) gene hnf4a[36] and the t2d gene cdkn2a[37] in sat had significant permutation adjusted p-values. |
| PubMedID- 24026554 | This was obtained from medical records or physician reports and categorized as type 1 (combining type 1, type 1a, and type 1b), type 2, secondary diabetes, and other types (such as “hybrid,” maturity-onset diabetes of the young, other type, and unknown/missing). |
| PubMedID- 21189947 | Mutations in the genes encoding hnf-1β produce an autosomal dominant disorder maturity-onset diabetes of the young, type 5 (mody5), also known as renal cysts and diabetes (rcad), because affected individuals present with severe cystic kidney disease40). |
| PubMedID- 25506511 | Mutations in hnf1β (also known as tcf2, previously discussed) have been associated with maturity-onset diabetes of the young renal dysfunction and müllerian aplasia [92]. |
| PubMedID- 24026547 | maturity-onset diabetes of the young (mody) is the most prevalent form of monogenic diabetes, all types of which account for 1–2% of diabetes cases (1). |
| PubMedID- 26311117 | In human, hnf-1b was first described to be associated with disease in 1997 that heterozygous germline mutations in hnf-1b cause maturity-onset diabetes of the young, subtype 5(mody5)5. in adults, hnf-1b is strongly expressed in the biliary system and is also expressed in the periportal hepatocytes6. |
| PubMedID- 21085603 | For example, rare highly penetrant sequence variants in several genes (hnf4a, gck, tcf1/hnf1a, tcf2/hnf1b) invariably cause a monogenic disorder known as maturity-onset diabetes of the young (mody), while common less penetrant variants in the same genes are risk factors for multifactorial type 2 diabetes. |
| PubMedID- 25806089 | As shown in additional file 8, three kegg pathways were found to be significantly enriched in clusters positively associated with age, including the hedgehog signaling pathway (adjusted p = 3.96 × 10−3; figure 5a) and the maturity-onset diabetes of the young (mody) pathway (adjusted p = 6.26 × 10−3; figure 5b) and neuroactive ligand-receptor interaction (adjusted p = 1.58 × 10−2). |
| PubMedID- 26384018 | Dominant genetic forms that become symptomatic during adolescence or early adulthood are also referred to as maturity-onset diabetes of the young (mody), while neonatal diabetes generally manifests in the first 6 months of life, and can result from dominant as well as recessive mutations24. |
| PubMedID- 22837374 | Patients with maturity-onset diabetes of the young, type 2 diabetes, or secondary diabetes were excluded. |
| PubMedID- 21949805 | The intracellular signaling mechanisms regulating insulin secretion have been extensively studied [4], and transcription factors have been linked to β-cell dysfunction in type 2 diabetes and maturity-onset diabetes of the young [3], [5]. |
| PubMedID- 26464794 | Mutations in the hnf1β gene cause maturity-onset diabetes of the young type 5 (mody5), renal cysts, genital malformations, and pancreas atrophy. |
| PubMedID- 24647409 | We have induced severe diabetes in pigs carrying a dominant-negative mutant hepatocyte nuclear factor 1-alpha (hnf1α) p291fsinsc, a maturity-onset diabetes of the young type-3 (mody3) gene in humans. |
| PubMedID- 26239553 | Ipsc lines have so far been generated from patients with type 1 and type 2 diabetes, as well as maturity-onset diabetes of the young [36,37,38]. |
| PubMedID- 23071669 | Hnf4a, a nuclear receptor transcription factor, has been linked to developmental and metabolic functions, and to several diseases, including maturity-onset diabetes of the young and type 2 diabetes [50]. |
| PubMedID- 23346354 | Our data is consistent with clinical investigation, in which a dramatic reduction in sp1 binding to gck promoter sequence corresponds to gck-mody (maturity-onset diabetes of the young) cases33. |
| PubMedID- 23942200 | Other topics, such as cardiogenetics (ie, long qt syndrome or hyperthrophic cardiomyopathy) or diabetes (ie, maturity-onset diabetes of the young), could also be trained in this framework. |
| PubMedID- 20007936 | Here, we sought to identify ins mutations associated with maturity-onset diabetes of the young (mody) or nonautoimmune diabetes in mid-adult life, and to explore the molecular mechanisms involved. |
| PubMedID- 21193628 | They are referred to as maturity-onset diabetes of the young (mody) and are characterized by impaired insulin secretion with minimal or no defects in insulin action. |
| PubMedID- 23803251 | Hnf1a: hepatocyte nuclear factor-1alpha; hnf4a: hepatocyte nuclear factor-4alpha; mody: maturity-onset diabetes of the young; psp/reg: pancreatic stone protein / regenerating protein; hscrp: high-sensitivity c-reactive protein; dn: dominant-negative; elisa: enzyme-linked immunosorbent assay; glut-2: glucose transporter 2; ins-1: insulinoma cell line-1; roc: receiver operating characteristic (roc); lda: linear discriminant analysis. |
| PubMedID- 23542897 | Common variants in several neonatal diabetes mellitus and maturity-onset diabetes of the young (mody) [e.g., potassium inwardly rectifying channel, subfamily j, member 11 (kcnj11), glucokinase (gck), hepatocyte nuclear factor 4 alpha (hnf1α), and hnf1β] are recognized as type 2 diabetes susceptibility variants 6 and 43 (bonnefond et al., 2010). |
| PubMedID- 25790815 | An association with maturity-onset diabetes of the young (mody), type 3 and familial hepatic adenomatosis has been reported [36, 43, 44]. |
| PubMedID- 25802718 | Thus, heterozygous inactivating mutations cause familial, mild fasting hyperglycemia, also known as maturity-onset diabetes of the young type 2 (mody2). |
| PubMedID- 26139844 | 2014), maturity-onset diabetes of the young (johansson et al. |
| PubMedID- 23761103 | Monogenic diabetes, or maturity-onset diabetes of the young (mody), is caused by mutations of these genes (hnf1α-mody, hnf1β-mody, pdx1-mody, neurod1-mody) (11–16). |
| PubMedID- 23342076 | Subjects who tested positive for anti-glutamic acid decarboxylase (gad) antibodies and those diagnosed with mitochondrial disease (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes [melas]) or maturity-onset diabetes of the young (mody) were not included. |
| PubMedID- 21788644 | Although tattersall recognized that these patients “appeared to have maturity-onset type diabetes at an unusually early age,” he did not use the term “maturity-onset diabetes of the young” (12). |
| PubMedID- 20181263 | Patients with clinical or autoimmune type 1 diabetes and families with known maturity-onset diabetes of the young or mitochondrial dna nucleotide 3243 a > g mutations were excluded. |
| PubMedID- 24669230 | Patients with type 1, gestational, or maturity-onset diabetes of the young were excluded. |
| PubMedID- 23033245 | We planned to exclude any autoantibody-negative children with diabetes occurring in three successive generations as suspected mody (maturity-onset diabetes of the young) cases. |
| PubMedID- 25598768 | Using wes in the maturity-onset diabetes of the young (mody) patients, the same research group found one mutation (p.glu227lys) in kcnj11, indicating that such mody patients can be ideally treated with oral sulfonylureas [51]. |
| PubMedID- 20936101 | maturity-onset diabetes of the young (mody) is a rare, autosomal dominant form of diabetes. |
| PubMedID- 21700917 | maturity-onset diabetes of the young (mody) is a rare monogenic form of diabetes and is often misdiagnosed as type 1 diabetes or type 2 diabetes (1,2). |
| PubMedID- 25621134 | The following were excluded: (i) patients with diabetes undergoing thiazolidinedione therapy; (ii) patients with diagnosed diabetic nephropathy or neuropathy; (iii) patients with acute or chronic inflammatory disease; and (iv) patients with type 1 diabetes, maturity-onset diabetes of the young or mitochondrial diabetes. |
| PubMedID- 21324932 | For example, point mutations in the gck gene give rise to maturity-onset diabetes of the young 2 (gck-mody2) in humans and to a similar phenotype in the mouse (e.g. |
| PubMedID- 24497937 | Among these pathways, six (glycolysis/gluconeogenesis, fatty acid metabolism, tyrosine metabolism, pyrimidine metabolism, peroxisome and maturity-onset diabetes of the young) were focused on metabolic pathways. |
| PubMedID- 22618775 | Support for this hypothesis comes from studies on maturity-onset diabetes of the young (mody), a group of autosomal-dominant disorders that typically manifest in adolescents, predominantly resulting from heterozygous mutations in islet developmental transcription factors (hnf4α, hnf1α, hnf1β, ipf1/pdx1, neurod1, and perhaps others) (62). |
| PubMedID- 24734255 | The maturity-onset diabetes of the young (mody) is a familial monogenic form of early-onset type 2 diabetes, which usually develops in childhood, adolescence, or young adulthood [105]. |
| PubMedID- 26404250 | Obesity, vascular diseases, elevated androgen levels, tobacco, and alcohol abuse, as well as syndromic diseases (mccune-albright syndrome, glycogen storage disease type 1a, and maturity-onset diabetes of the young (mody) type 3, familiar adenomatous polyposis (fap)) add to the known risk factors for hca [2,3,4] (figure 1). |
| PubMedID- 25723573 | In addition to cell-compartmental differences, pathways/processes such as “maturity-onset diabetes of the young (kegg pathways)”, “adipocytokine signalling pathway (kegg pathways)”, and processes involved in pancreas biology and development were found to be significant for the production of sappα but not for sappβ (see s10 supplementary information). |
| PubMedID- 23846812 | Subjects also were excluded if they were 1) taking any medications known to influence body composition, insulin action, or insulin secretion (e.g., prednisone, ritalin, growth hormone); 2) diagnosed with syndromes or diseases that may influence insulin action and secretion (e.g., maturity-onset diabetes of the young, lipoatrophic diabetes, cystic fibrosis) or body composition and fat distribution (e.g., cushing syndrome, down syndrome); or, 3) previously diagnosed with any major illness since birth (e.g., severe intrauterine growth retardation, birth asphyxia, cancer) or a condition that could affect body composition, fat distribution, or insulin action or secretion. |
| PubMedID- 22187472 | They are referred to as maturity-onset diabetes of the young (mody) and are characterized by impaired insulin secretion with minimal or no defects in insulin action. |
| PubMedID- 20682686 | maturity-onset diabetes of the young (mody) is a familial form of non–insulin-dependent diabetes characterized by early onset of disease, autosomal dominant inheritance, and insulin secretory defects (1). |
| PubMedID- 20009086 | Homozygous or compound heterozygous mutations in pdx1 have been previously reported in two unrelated patients with neonatal diabetes and exocrine pancreas deficiency due to pancreas agenesis or hypoplasia (10,11), and heterozygous mutations are responsible for maturity-onset diabetes of the young (mody4) (7,12). |