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  • 1
    Keywords: World Trade Organization., Ministerial Conference, (5th :, 2003 :, Cancun, Mexico) ; Business. ; COMERCIALIZACION., renib ; Commercial policy. ; Commercial policy. ; Diplomatic negotiations in international disputes. ; Econometric models. ; Export subsidies. ; Free trade. ; LIBRE COMERCIO., renib ; MODELOS ECONOMETRICOS., renib ; Marketing. ; NEGOCIACIONES DIPLOMATICAS., renib ; NEGOCIOS., renib ; POLITICA COMERCIAL., renib ; POLITICA COMERCIAL., renib ; Treaties, Accession. ; Agreement on Agriculture (1994)
    Pages: vi, 46 p.
    ISBN: 1-417-57703-7
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  • 2
    Unknown
    Cambridge [England] ; New York : Cambridge University Press
    Keywords: Combustion engineering. ; Turbulence.
    Pages: xvi, 304 p.
    ISBN: 0-511-01927-0
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  • 3
    Call number: 04-ZELL:290c ; ATV-IMM:121(2) ; ATV-IMM:121(2)
    Type of Medium: Book
    Pages: xix, 484 p. : , ill.
    Edition: 2., überarb. u. erw. Aufl.
    ISBN: 3-540-50844-9
    Language: German
    Location: DKFZ
    Location: DKFZ
    Location: DKFZ
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  • 4
    Book
    Book
    Washington, D.C. : : Armed Forces Institute of Pathology : [Supt. of Docs., U.S. G.P.O., distributor] : For sale by the Armed Forces Institute of Pathology,
    Call number: QZ241:007/26
    Type of Medium: Book
    Pages: 280 p. : , ill. (some col.)
    Series Statement: Atlas of tumor pathology.
    Language: English
    Location: DKFZ
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  • 5
    Call number: QP501Z:003/50/Mag
    Type of Medium: Book
    Pages: vi, 79 p. : , ill. ; , 23 cm.
    ISBN: 0080226310 :
    Series Statement: Proceedings of the 11th FEBS meeting ; v. [9] 50
    Language: English
    Location: DKFZ
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  • 6
    Call number: E130:071
    Type of Medium: Audiovisual Media
    Pages: xxxii, 557 p : , ill. + , 1 DVD (4 3/4 in.).
    ISBN: 9780387738567 (hbk. : alk. paper) , 0387738568 (hbk. : alk. paper) , 9780387738581 (e-book)
    Language: English
    Location: DKFZ
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  • 7
    Online Resource
    Online Resource
    Berlin, Heidelberg : : Springer Berlin, Heidelberg, | Berlin, Heidelberg : : Springer Berlin Heidelberg :
    In: Springer eBooks
    Type of Medium: Online Resource
    Pages: VI, 112 p. 28 illus., 14 illus. in color. , online resource.
    Edition: 1st ed. 2016.
    ISBN: 9783662504093
    Series Statement: JIMD Reports, 27
    Language: English
    Note: Detailed Biochemical and Bioenergetic Characterization of FBXL4-Related Encephalomyopathic Mitochondrial DNA Depletion -- Recurrent Ventricular Tachycardia in Medium-Chain Acyl-Coenzyme A Dehydrogenase Deficiency -- Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts from Patients with Inherited Disorders -- SUCLA2〈 Deficiency: A Deafness-Dystonia Syndrome with Distinctive Metabolic Findings (Report of a New Patient and Review of the Literature) -- Diagnostic Value of Urinary Mevalonic Acid Excretion in Patients with a Clinical Suspicion of Mevalonate Kinase Deficiency (MKD) -- Hyperprolinemia in Type 2 Glutaric Aciduria and MADD-Like Profiles -- IgG N-Glycosylation Galactose Incorporation Ratios for the Monitoring of Classical Galactosaemia -- Intracranial Pressure Monitoring Demonstrates that Cerebral Edema Is Not Correlated to Hyperammonemia in a Child with Ornithine Transcarbamylase Deficiency -- No Evidence for Association of SCO2 Heterozygosity with High-Grade Myopia or Other Diseases with Possible Mitochondrial Dysfunction -- Voluntary Exercise Prevents Oxidative Stress in the Brain of Phenylketonuria Mice -- Seizures Due to a KCNQ2 Mutation: Treatment with Vitamin B6 -- The Frequencies of Different Inborn Errors of Metabolism in Adult Metabolic Centres: Report from the SSIEM Adult Metabolic Physicians Group -- Electroclinical Features of Early-Onset Epileptic Encephalopathies in Congenital Disorders of Glycosylation (CDGs) -- The Newborn Screening Paradox: Sensitivity vs. Overdiagnosis in VLCAD Deficiency -- Further Delineation of the ALG9-CDG Phenotype.
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  • 8
    Online Resource
    Online Resource
    Berlin, Heidelberg : : Springer, | Berlin, Heidelberg : : Springer Berlin Heidelberg :
    In: Springer eBooks
    Type of Medium: Online Resource
    Pages: VI, 113 p. 34 illus., 14 illus. in color. , online resource.
    Edition: 1st ed. 2016.
    ISBN: 9783662532782
    Series Statement: JIMD Reports,
    Language: English
    Note: LC-MS/MS Analysis of Cerebrospinal Fluid Metabolites in the Pterin Biosynthetic Pathway -- Renal Involvement in a French Paediatric Cohort of Patients with Lysinuric Protein Intolerance -- A Highly Diverse Portrait: Heterogeneity of Neuropsychological Profiles in cblC Defect -- Heterozygous Monocarboxylate Transporter 1 (MCT1, SLC16A1) Deficiency as a Cause of Recurrent Ketoacidosis -- Spectrum of Mutations in 60 Saudi Patients with Mut Methylmalonic Acidemia -- CoQ10 Deficiency Is Not a Common Finding in GLUT1 Deficiency Syndrome -- Correlation Between Flexible Fiberoptic Laryngoscopic and Polysomnographic Findings in Patients with Mucopolysaccharidosis Type VI -- Continual Low-Dose Infusion of Sulfamidase Is Superior to Intermittent High-Dose Delivery in Ameliorating Neuropathology in the MPS IIIA Mouse Brain -- A Short Synthetic Peptide Mimetic of Apolipoprotein A1 Mediates Cholesterol and Globotriaosylceramide Efflux from Fabry Fibroblasts -- Development of Metabolic Phenotype in Phenylketonuria: Evaluation of the Blaskovics Protein Loading Test at 5 Years of Age -- The Lactose and Galactose Content of Cheese Suitable for Galactosaemia: New Analysis -- Atypical Clinical Presentations of TAZ Mutations: An Underdiagnosed Cause of Growth Retardation? -- Long-Term Cognitive and Functional Outcomes in Children with Mucopolysaccharidosis (MPS)-IH (Hurler Syndrome) Treated with Hematopoietic Cell Transplantation -- Treatment with Mefolinate (5-Methyltetrahydrofolate), but Not Folic Acid or Folinic Acid, Leads to Measurable 5-Methyltetrahydrofolate in Cerebrospinal Fluid in Methylenetetrahydrofolate Reductase Deficiency -- Abnormal Glycosylation Profile and High Alpha-Fetoprotein in a Patient with Twinkle Variants -- Erratum to: LC-MS/MS Analysis of Cerebrospinal Fluid Metabolites in the Pterin Biosynthetic Pathway -- Erratum to: Treatment with Mefolinate (5-Methyltetrahydrofolate), but Not Folic Acid or Folinic Acid, Leads to Measurable 5-Methyltetrahydrofolate in Cerebrospinal Fluid in Methylenetetrahydrofolate Reductase Deficiency.
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  • 9
    Online Resource
    Online Resource
    Berlin, Heidelberg : : Springer International Publishing, | Berlin, Heidelberg : : Springer Berlin Heidelberg :
    In: Springer eBooks
    Type of Medium: Online Resource
    Pages: VI, 108 p. 20 illus., 13 illus. in color. , online resource.
    Edition: 1st ed. 2016.
    ISBN: 9783662536810
    Series Statement: JIMD Reports, 30
    Language: English
    Note: Multidisciplinary Team Approach Is Key for Managing Pregnancy and Delivery in Patient with Rare, Complex MPS I -- Clinical Evolution After Enzyme Replacement Therapy in Twins with the Severe Form of Maroteaux–Lamy Syndrome -- A New Approach for Fast Metabolic Diagnostics in CMAMMA -- Pilot Experience with an External Quality Assurance Scheme for Acylcarnitines in Plasma/Serum -- ECHS1 Deficiency as a Cause of Severe Neonatal Lactic Acidosis -- Chronic Oral l-Carnitine Supplementation Drives Marked Plasma TMAO Elevations in Patients with Organic Acidemias Despite Dietary Meat Restrictions -- A Founder Effect for the HGD G360R Mutation in Italy: Implications for a Regional Screening of Alkaptonuria -- Missed Newborn Screening Case of Carnitine Palmitoyltransferase-II Deficiency -- Acute Metabolic Crises in Maple Syrup Urine Disease After Liver Transplantation from a Related Heterozygous Living Donor -- Identification of Cryptic Novel α-Galactosidase A Gene Mutations: Abnormal mRNA Splicing and Large Deletions -- Severe Neonatal Presentation of Mitochondrial Citrate Carrier (SLC25A1) Deficiency -- Biomarkers in a Taurine Trial for Succinic Semialdehyde Dehydrogenase Deficiency -- A Modified Enzymatic Method for Measurement of Glycogen Content in Glycogen Storage Disease Type IV -- The Effect of Multiple Sulfatase Deficiency (MSD) on Dental Development: Can We Use the Teeth as an Early Diagnostic Tool? -- Novel Report of Phosphoserine Phosphatase Deficiency in an Adult with Myeloneuropathy and Limb Contractures -- Erratum to: Novel Report of Phosphoserine Phosphatase Deficiency in an Adult with Myeloneuropathy and Limb Contractures.
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  • 10
    Online Resource
    Online Resource
    Berlin, Heidelberg : : Springer, | Berlin, Heidelberg : : Springer Berlin Heidelberg :
    In: Springer eBooks
    Type of Medium: Online Resource
    Pages: VI, 124 p. 30 illus., 15 illus. in color. , online resource.
    ISBN: 9783662543856
    Series Statement: JIMD Reports, 32
    Language: English
    Note: Establishing New Cut-Off Limits for Galactose 1-Phosphate-Uridyltransferase Deficiency for the Dutch Newborn Screening Programme -- Four Years of Diagnostic Challenges with Tetrahydrobiopterin Deficiencies in Iranian Patients -- Endurance Exercise Training in Young Adults with Barth Syndrome: A Pilot Study -- Hydroxysteroid 17-Beta Dehydrogenase Type 10 Disease in Siblings -- Reliable Diagnosis of Carnitine Palmitoyltransferase Type IA Deficiency by Analysis of Plasma Acylcarnitine Profiles -- Relationships Between Childhood Experiences and Adulthood Outcomes in Women with PKU: A Qualitative Analysis -- The Effect of S-Adenosylmethionine on Self-Mutilation in a Patient with Lesch–Nyhan Disease -- Low Protein Formula: Consequences of Quantitative Effects of Pre-analytical Factors on Amino Acid Concentrations in Plasma of Healthy Infants -- A Multiplatform Metabolomics Approach to Characterize Plasma Levels of Phenylalanine and Tyrosine in Phenylketonuria -- Japanese Male Siblings with 2-Methyl-3-Hydroxybutyryl-CoA Dehydrogenase Deficiency (HSD10 Disease) Without Neurological Regression -- Newborn Screening for Vitamin B6 Non-responsive Classical Homocystinuria: Systematical Evaluation of a Two-Tier Strategy -- Management of an LCHADD Patient During Pregnancy and High Intensity Exercise -- Rare Case of Hepatic Gaucheroma in a Child on Enzyme Replacement Therapy -- Newborn Screening Programmes in Europe, Arguments and Efforts Regarding Harmonisation: Focus on Organic Acidurias -- Whole Exome Sequencing Identifies the Genetic Basis of Late-Onset Leigh Syndrome in a Patient with MRI but Little Biochemical Evidence of a Mitochondrial Disorder.
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