Research Topics
Genomes and Genes | Ina KnerrSummaryAffiliation: University of Erlangen-Nuremberg Country: Germany Publications
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Publications
Endocrine effects of food intake: insulin, ghrelin, and leptin responses to a single bolus of essential amino acids in humansI Knerr
Department of Paediatrics, University of Erlangen Nuremberg, Loschgestrasse 15, DE 91054 Erlangen, Germany
Ann Nutr Metab 47:312-8. 2003..This study deals with the physiological effects of an oral bolus of essential amino acids (AA) on the secretion of insulin, ghrelin and leptin in fasting humans...- Fusiogenic endogenous-retroviral syncytin-1 exerts anti-apoptotic functions in staurosporine-challenged CHO cellsIna Knerr
Children and Youth Hospital, University of Erlangen Nuremberg, Loschgestr 15, D 91054, Erlangen, Germany
Apoptosis 12:37-43. 2007..Our study provides first evidence that syncytin-1 serves anti-apoptotic function under certain conditions. A lessened activation of caspase 3 and an increased expression of Bcl-2 are possible mechanisms... - Leptin and ghrelin expression in adipose tissues and serum levels in gastric banding patientsI Knerr
Children and Youth Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Eur J Clin Invest 36:389-94. 2006..To determine how leptin and ghrelin are expressed in the adipose tissues of obese adults undergoing gastric banding (LAGB), and to correlate tissue expression with serum concentrations and parameters of the metabolic syndrome... - Transcriptional effects of hypoxia on fusiogenic syncytin and its receptor ASCT2 in human cytotrophoblast BeWo cells and in ex vivo perfused placental cotyledonsIna Knerr
Department of Pediatrics, University of Erlangen Nuremberg, Erlangen, Germany
Am J Obstet Gynecol 189:583-8. 2003.... - Laparoscopically performed gastric banding in a 13 year-old girl with morbid obesity and end stage renal insufficiency allows lifesaving weight loss and hemodialysisI Knerr
Department of Pediatrics, University of Erlangen Nuremberg, Germany
J Pediatr Endocrinol Metab 16:1179-82. 2003..However, little information is available on gastric banding in children... - Hematologic features and clinical course of an infant with Pearson syndrome caused by a novel deletion of mitochondrial DNAIna Knerr
Department of Pediatrics, University of Erlangen Nuremberg, Germany
J Pediatr Hematol Oncol 25:948-51. 2003..371 to 14.607... - The 'accelerator hypothesis': relationship between weight, height, body mass index and age at diagnosis in a large cohort of 9,248 German and Austrian children with type 1 diabetes mellitusI Knerr
University Children s Hospital, University of Erlangen Nuremberg, Loschgestr 15, 91054, Erlangen, Germany
Diabetologia 48:2501-4. 2005..The aim of this study was to investigate whether either increased weight or BMI are associated with the earlier manifestation of type 1 diabetes mellitus in children... - An exceptional Albanian family with seven children presenting with dysmorphic features and mental retardation: maternal phenylketonuriaIna Knerr
Children and Youth Hospital, University of Erlangen Nuremberg, Loschge Street 15, 91054 Erlangen, Germany
BMC Pediatr 5:5. 2005..The maternal phenylketonuria syndrome is caused by high blood phenylalanine concentrations during pregnancy and presents with serious foetal anomalies, especially congenital heart disease, microcephaly and mental retardation... - Maturation of the expression of adrenomedullin, endothelin-1 and nitric oxide synthases in adipose tissues from childhood to adulthoodI Knerr
University Children s Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Int J Obes (Lond) 29:275-80. 2005..To investigate if the vasoactive systems adrenomedullin (ADM) and endothelin-1 (ET-1) are expressed in human adipose tissues in children and in adults and to determine the distribution pattern of nitric oxide synthases (NOS)... - Glutaric aciduria type III: a distinctive non-disease?I Knerr
Department of Paediatrics, University of Erlangen Nuremberg, Erlangen, Germany
J Inherit Metab Dis 25:483-90. 2002..Glutaric aciduria type III appears to be a rare metabolic abnormality, presumably of peroxisomal metabolism. However, its pathophysiological impact still needs further investigation... - Prevailing therapeutic regimes and predictive factors for prandial insulin substitution in 26 687 children and adolescents with Type 1 diabetes in Germany and AustriaI Knerr
University Children s Hospital, University of Erlangen Nuremberg, Germany
Diabet Med 24:1478-81. 2007..To analyse current therapeutic strategies for prandial insulin substitution in a large number of children and adolescents with Type 1 diabetes in Germany and Austria, along with changes in therapeutic habits and outcome... - [Early factors influencing body weight and prevalence of overweight in 4610 children prior to school entry in the Erlangen District (Northern Bavaria)]I Knerr
Kinder und Jugendklinik des Universitätsklinikums Erlangen, Friedrich Alexander Universitat Erlangen Nurnberg
Gesundheitswesen 67:183-8. 2005..We tested other parameters influencing childhood obesity, and hypothesized that within a 5-year interval the prevalence of obesity in children has increased significantly... - Adrenomedullin, calcitonin gene-related peptide and their receptors: evidence for a decreased placental mRNA content in preeclampsia and HELLP syndromeIna Knerr
Department of Pediatrics, University of Erlangen Nuremberg, Erlangen, Germany
Eur J Obstet Gynecol Reprod Biol 101:47-53. 2002..These are severe maternal conditions leading to an altered uteroplacental and fetoplacental perfusion and a higher risk for foetal growth retardation, premature delivery, infant mortality, and even maternal death... - Stimulation of GCMa and syncytin via cAMP mediated PKA signaling in human trophoblastic cells under normoxic and hypoxic conditionsIna Knerr
University Hospital for Children and Adolescents, University of Erlangen Nuremberg, Germany
FEBS Lett 579:3991-8. 2005..We propose that GCMa-driven syncytin expression is the key mechanism for syncytiotrophoblast formation... - Differential regulation of leptin synthesis in rats during short-term hypoxia and short-term carbon monoxide inhalationUdo Meissner
Department of Pediatrics, University of Erlangen Nurnberg, 91054 Erlangen, Germany
Endocrinology 146:215-20. 2005..Inhalation of CO leads to a significant decrease in leptin mRNA and protein concentration in the plasma of the animals, suggesting a negative effect of CO on leptin transcription... - Childhood obesity: concept, feasibility, and interim results of a local group-based, long-term treatment programCorina Weigel
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Germany
J Nutr Educ Behav 40:369-73. 2008..The authors compared this program with a control approach based on the patients' own initiative... - Response of HEK293 and CHO cells overexpressing fusiogenic syncytin-1 to mitochondrion-mediated apoptosis induced by antimycin AIna Knerr
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
J Cell Biochem 105:766-75. 2008..A reduced release of cytochrome c followed by a diminished activation of caspase 9 is a possible mechanism... - Leptin expression in primary trophoblast cells in response to incubation with the serum of preeclamptic womenRobert M Spranger
Department of Pediatrics and Adolescent Medicine, University of Erlangen Nuernberg, Erlangen, Germany
Hypertens Pregnancy 27:267-77. 2008..We investigated whether the increase of leptin expression in preeclamptic placentas is additionally influenced by soluble maternal factors under hypoxic and nonhypoxic conditions... - Tracking and prediction of arterial blood pressure from childhood to young adulthood in 868 patients with type 1 diabetes: a multicenter longitudinal survey in Germany and AustriaIna Knerr
Children s and Adolescents Hospital, University of Erlangen Nuremberg, D 91054 Erlangen, Germany
Diabetes Care 31:726-7. 2008..Arterial blood pressure was followed in 868 patients with type 1 diabetes aged 6.0-19.9 years in 95 centers in Germany and Austria... - Endocrine responses to the oral ingestion of a physiological dose of essential amino acids in humansM Groschl
Department of Paediatrics, Friedrich Alexander Universität Erlangen Nuremberg, Germany
J Endocrinol 179:237-44. 2003..The endocrine data provided in this study indicate that a single bolus of essential AA in fasted individuals is associated with both anabolic and catabolic hormonal responses... - Syncytin, a novel human endogenous retroviral gene in human placenta: evidence for its dysregulation in preeclampsia and HELLP syndromeIna Knerr
Department of Pediatrics, University of Erlangen Nuremberg, Germany
Am J Obstet Gynecol 186:210-3. 2002..We tested the hypothesis of whether the gene expression of syncytin may be altered in cases with placental dysfunction such as preeclampsia or HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome... - Nitric oxide increases adrenomedullin receptor function in rat mesangial cellsJorg Dotsch
Klinik fur Kinder und Jugendliche, and Medizinische Klinik IV, Friedrich Alexander University Erlangen, Erlangen, Germany
Kidney Int 61:1707-13. 2002..In contrast, nitric oxide (NO) is capable of exerting both cytoprotective and cytotoxic actions. It was the objective of the present study to examine whether NO stimulates the ADM system... - Increase of endothelial nitric oxide synthase and endothelin-1 mRNA expression in human placenta during gestationJ Dotsch
Department of Pediatrics, University of Erlangen Nurnberg, Loschgestrasse 15, 91054, Erlangen, Germany
Eur J Obstet Gynecol Reprod Biol 97:163-7. 2001..To investigate the maturation of the paracrine system's endothelial nitric oxide synthase (eNOS), inducible NOS (iNOS), endothelin-1 (ET-1) and adrenomedullin (AM) in human placenta during the 2nd and 3rd trimester of pregnancy... - Expression of neuropeptide Y, omentin and visfatin in visceral and subcutaneous adipose tissues in humans: relation to endocrine and clinical parametersStephanie Barth
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Obes Facts 3:245-51. 2010..We aimed at exploring the expression of neuropeptide Y (NPY), omentin and visfatin in adipose tissues of adults along with clinical parameters and hormones... - Severe, neonatal-onset OTC deficiency in twin sisters with a de novo balanced reciprocal translocation t(X;5)(p21.1;q11)Martin Zenker
Institute of Human Genetics, Friedrich Alexander University Erlangen Nuremberg, Germany
Am J Med Genet A 132:185-8. 2005..This report emphasizes the importance of chromosome analysis in any female manifesting severe OTC deficiency... - Two novel mutations in the insulin binding subunit of the insulin receptor gene without insulin binding impairment in a patient with Rabson-Mendenhall syndromeChristian T Thiel
Institute of Human Genetics, Friedrich Alexander University of Erlangen Nuremberg, Schwabachanlage 10, D 91054 Erlangen, Germany
Mol Genet Metab 94:356-62. 2008..Our data indicate that the quality of insulin binding activity is correlated with survival, not with the dysmorphic phenotype, and it is not always a valid parameter for predicting INSR mutations as proposed... - Homocysteine concentrations in a German cohort of 500 individuals: reference ranges and determinants of plasma levels in healthy children and their parentsM Rauh
University Children s Hospital, Friedrich Alexander University, Erlanen Nuermberg, Erlangen, Federal Republic of Germany
Amino Acids 20:409-18. 2001..Genetic and nutritional factors are determinants of Hcys metabolism. The correlation of Hcys and serum creatinine is dependent on the metabolic link between Hcys production and creatine synthesis... - Endogenous retroviral syncytin: compilation of experimental research on syncytin and its possible role in normal and disturbed human placentogenesisI Knerr
University Children s Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Mol Hum Reprod 10:581-8. 2004..We evaluate the hypothesis that syncytin is essential for human placenta formation and may also have played an important role in human placental evolution... - Effects of various dietary amino acid preparations for phenylketonuric patients on the metabolic profiles along with postprandial insulin and ghrelin responsesC Weigel
Children and Youth Hospital, University of Erlangen Nurnberg, Erlangen, Germany
Ann Nutr Metab 51:352-8. 2007..We investigated the metabolic profiles along with insulin and ghrelin responses following ingestion of various amino acid (AA) substitutes commonly used in the treatment of phenylketonuria to study the effects of added macronutrients... - Does defective hypoxia signalling cause type 2 diabetes?Jorg Dotsch
Department of Pediatrics, Friedrich Alexander University Erlangen Nuremberg, Loschgestrasse 15, 91054 Erlangen, Germany
Eur J Endocrinol 154:781-2. 2006 - Transient insulin-dependent diabetes mellitus in children with steroid-dependent idiopathic nephrotic syndrome during tacrolimus treatmentKatalin Dittrich
Children and Adolescent Hospital, , Loschgestrasse 15, 91054, Erlangen, Germany
Pediatr Nephrol 21:958-61. 2006..Moreover, Tac does not appear to substantially increase the success of treatment... - Syncytin-1 and glial cells missing a: hypoxia-induced deregulated gene expression along with disordered cell fusion in primary term human trophoblastsC Wich
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Gynecol Obstet Invest 68:9-18. 2009..We investigated how different levels of hypoxia alter the expression of syncytin-1, glial cells missing a (GCMa) and syncytin-1 receptor ASCT2 and affect syncytialization in primary term human trophoblasts... - Diagnostic challenges in a severely delayed infant with hypersomnolence, failure to thrive and arteriopathy: a unique case of gamma-hydroxybutyric aciduria and Williams syndromeIna Knerr
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Am J Med Genet B Neuropsychiatr Genet 144:946-8. 2007.... - Leptin and neuropeptide Y gene expression in human placenta: ontogeny and evidence for similarities to hypothalamic regulationJ Dotsch
Universitätsklinik für Kinder und Jugendliche, Erlangen, Germany
J Clin Endocrinol Metab 84:2755-8. 1999..Our data suggest an increase of placental leptin production with gestational age. In patients with preeclampsia, elevated leptin expression goes along with suppressed NPY expression. This resembles hypothalamic regulation... - Increased endothelin-1 and decreased adrenomedullin gene expression in the stenotic tissue of congenital pelvi-ureteric junction obstruction in childrenI Knerr
Department of Paediatrics, Friedrich-Alexander University, Erlangen, Germany
BJU Int 87:667-71. 2001.... - Clinical, cytogenetic and molecular characterization of a patient with combined succinic semialdehyde dehydrogenase deficiency and incomplete WAGR syndrome with obesityRonny Jung
Children and Youth Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Mol Genet Metab 88:256-60. 2006..In summary, we report the first patient with the exceptional rare findings of both SSADH deficiency and partial WAGR/WAGRO syndrome... - Levothyroxine in euthyroid autoimmune thyroiditis and type 1 diabetes: a randomized, controlled trialBeate Karges
Division of Pediatric Endocrinology and Diabetes, University Children s Hospital, University of Ulm, Eythstrasse 24, D 89075 Ulm, Germany
J Clin Endocrinol Metab 92:1647-52. 2007..Patients with type 1 diabetes (T1D) have an increased risk of autoimmune thyroiditis (AIT)... - Course of placental 11beta-hydroxysteroid dehydrogenase type 2 and 15-hydroxyprostaglandin dehydrogenase mRNA expression during human gestationE Schoof
Department of Pediatrics, University of Erlangen-Nuremberg, Germany
Eur J Endocrinol 145:187-92. 2001..11beta-HSD2 up-regulation may also lead to an increase in PGDH mRNA concentrations that, until term, possibly delays myometrial contractions induced by prostaglandins... - Alteration of neuronal and endothelial nitric oxide synthase and neuropeptide Y in congenital ureteropelvic junction obstructionI Knerr
Department of Pediatrics, , Germany
Urol Res 29:134-40. 2001..CONCLUSION: Taken together, we found alterations in NOS gene expression and NPY innervation in tissue specimens of patients with congenital ureteropelvic junction obstruction... - Carnitine status in early-treated children, adolescents and young adults with phenylketonuria on low phenylalanine dietsC Weigel
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
Ann Nutr Metab 53:91-5. 2008..In patients with phenylketonuria (PKU), the carnitine status may be impaired for metabolic or dietary reasons, including low carnitine intake, a deficient synthesis and acylcarnitine production from phenylalanine (Phe) metabolites... - Therapeutic concepts in succinate semialdehyde dehydrogenase (SSADH; ALDH5a1) deficiency (gamma-hydroxybutyric aciduria). Hypotheses evolved from 25 years of patient evaluation, studies in Aldh5a1-/- mice and characterization of gamma-hydroxybutyric acid I Knerr
Children s and Adolescents Hospital, University of Erlangen Nuremberg, Erlangen, Germany
J Inherit Metab Dis 30:279-94. 2007.... - Reduced frequency of peripheral plasmacytoid dendritic cells in type 1 diabetesC Hinkmann
Department of Medicine 1, Friedrich Alexander University Erlangen Nurnberg, Erlangen, Germany
Horm Metab Res 40:767-71. 2008..Since pDCs are involved in the control of immune responses and inducing regulatory cells, a reduced number of pDCs may predispose to an autoimmune reaction in the pancreatic islets... - Congenital unilateral ureteropelvic junction obstruction of the rat: a useful animal model for human ureteropelvic junction obstruction?Joerg Miller
Department of Urology, Justus Liebig University of Giessen, Giessen, Germany
Urology 63:190-4. 2004..Our study group has recently been able to demonstrate a significant increase in ET-1 and a significant decrease in ADM in prestenotic and stenotic tissue, but not in the remainder of the ureter, compared with controls... - Characterization of monocyte-derived dendritic cells in recent-onset diabetes mellitus type 1Thorsten Zacher
Institute for Clinical Immunology, Department of Medicine III, University of Erlangen-Nuremberg, Krankenhausstrasse 12, D-91054 Erlangen, Germany
Clin Immunol 105:17-24. 2002..In conclusion, our analysis of a cohort of recent-onset DM-1 patients and controls does not support a role for disease-related alterations in cytokine-driven maturation of monocyte-derived DC... - [Obesity and the metabolic syndrome in children and adolescents]Ina Knerr
MMW Fortschr Med 146:41-3. 2004.... - Neuropsychiatric morbidity in adolescent and adult succinic semialdehyde dehydrogenase deficiency patientsIna Knerr
Department of Neurology, Children s National Medical Center, George Washington University School of Medicine, Washington, DC, USA
CNS Spectr 13:598-605. 2008..While neurological manifestations, such as developmental delay, are typical during infancy, limited data are available on adolescent and adult symptomatology... - Delayed menarche in young German women with type 1 diabetes mellitus: recent results from the DPV diabetes documentation and quality management systemTilman Rohrer
Department of Pediatric and Adolescent Medicine, Pediatric Diabetes Unit, Saarland University Hospital, Homburg, Saar, Germany
Eur J Pediatr 167:793-9. 2008..Findings have been inconsistent regarding the effect of T1DM (type 1 diabetes) on age at menarche... - bZIP-Type transcription factors CREB and OASIS bind and stimulate the promoter of the mammalian transcription factor GCMa/Gcm1 in trophoblast cellsSteffen Wolfgang Schubert
Institut fur Biochemie, Emil Fischer Zentrum, Kinder und Jugendklinik and Nikolaus Fiebiger Zentrum, Universitat Erlangen Nurnberg, Fahrstrasse 17, D 91054 Erlangen, Germany
Nucleic Acids Res 36:3834-46. 2008..Further, we show that CREB expression is replaced by OASIS expression around E12.5 suggesting that a sequential order of bZIP-type family members ensures a high rate of GCMa transcription throughout placentation... - Identification of 16 novel mutations in the argininosuccinate synthetase gene and genotype-phenotype correlation in 38 classical citrullinemia patientsHong Zhi Gao
Department of Biochemistry, Faculty of Medicine, Kagoshima University, Kagoshima, Japan
Hum Mutat 22:24-34. 2003.... - Newborn screening for 3-methylcrotonyl-CoA carboxylase deficiency: population heterogeneity of MCCA and MCCB mutations and impact on risk assessmentSonja C Stadler
Research Center, Department of Biochemical Genetics and Molecular Biology, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University, Munich, Germany
Hum Mutat 27:748-59. 2006..MCCD may be regarded as exemplary of the ongoing controversy arising from the inclusion of potentially asymptomatic conditions, which generates a psychological burden for afflicted families and a financial burden for health care systems... - Ketogenic diet: stoking energy stores and still posing questionsIna Knerr
Department of Neurology, Children's National Medical Center, George Washington University School of Medicine, Washington, DC 20010, USA
Exp Neurol 211:11-3. 2008 - Reduced incidence of severe metabolic crisis or death in children with medium chain acyl-CoA dehydrogenase deficiency homozygous for c.985A>G identified by neonatal screeningUta Nennstiel-Ratzel
Screening Center of the Bavarian Health and Food Safety Authority, Oberschleissheim, Germany
Mol Genet Metab 85:157-9. 2005..Logrank test showed a significant reduction of severe metabolic crises in the screened cohort (p<0.01). Neonatal screening appears to reduce the rate of severe metabolic crisis or death in the most prevalent subset of MCADD...