Page last updated: 2024-09-05

biotin and beta-hydroxyisovaleric acid

biotin has been researched along with beta-hydroxyisovaleric acid in 36 studies

Compound Research Comparison

Studies
(biotin)
Trials
(biotin)
Recent Studies (post-2010)
(biotin)
Studies
(beta-hydroxyisovaleric acid)
Trials
(beta-hydroxyisovaleric acid)
Recent Studies (post-2010) (beta-hydroxyisovaleric acid)
14,847953,882442134278

Research

Studies (36)

TimeframeStudies, this research(%)All Research%
pre-19907 (19.44)18.7374
1990's10 (27.78)18.2507
2000's14 (38.89)29.6817
2010's4 (11.11)24.3611
2020's1 (2.78)2.80

Authors

AuthorsStudies
Mock, DM; Mock, NI1
Jackson, H; Lankford, GL; Mock, DM; Mock, NI; Weintraub, ST1
Holme, E; Jacobson, CE; Kristiansson, B1
Mock, DM; Mock, NI; Weintraub, S1
Baker, H; Baswell, DL; Holman, RT; Mock, DM; Sweetman, L1
Erasmus, C; Mienie, LJ; Reinecke, CJ; Wadman, SK1
Bartlett, K; Leonard, JV; Ng, H1
Sweetman, L1
Berry, SA; Nyhan, WL; Thuy, LP; Tuchman, M1
Baumgartner, ER; Lehnert, W; Niederhoff, H; Suormala, T1
Bishop, WP; Malik, MI; Mock, DM; Mock, NI; Stumbo, PJ1
Mock, DM; Mock, NI; Stadler, DD; Stratton, SL1
Mock, DM; Stadler, DD1
Dyken, ME; Mock, DM1
Engelbrecht, V; Gärtner, J; Lenard, HG; Lohmeier, K; Schürmann, M; Wendel, U1
Lombard, KA; Mock, DM; Mock, NI; Nelson, RP1
Mock, DM1
Helm, RM; Mock, DM; Mock, NI; Simpson, P1
Mock, DM; Mock, NI; Quirk, JG1
Boylan, J; Cannon, MJ; Cederbaum, S; Cress, S; Jennings, ML; Mardach, R; Mock, DM; Roth, S; Wolf, B; Zempleni, J1
Carnell, N; Henrich, CL; Mock, DM; Mock, NI1
Aoki, Y; Baumgartner, ER; Duran, M; Muhle, H; Santer, R; Stephani, U; Suormala, T; Suzuki, Y; Yang, X1
Almashanu, S; Baumgartner, ER; Baumgartner, MR; Dantas, MF; Fowler, B; Friebel, D; Gebhardt, B; Giunta, C; Hoffmann, GF; Suormala, T; Valle, D1
Mock, DM; Sealey, WM; Stratton, SL; Teague, AM1
Fukui, T; Kumasaka, K1
Ebara, S; Fukui, T; Oguchi, K; Watanabe, T1
Bogusiewicz, A; Mock, DM; Mock, MM; Mock, NI; Stratton, SL; Wells, AM1
Ferreira, G; Weiss, WP; Willett, LB1
López-Rosas, I; Rodríguez-Fuentes, N; Román-Cisneros, G; Velázquez-Arellano, A1
Ebara, S; Fukui, T; Kimura, S; Nagai, Y; Taniguchi, A; Watanabe, T1
Barshop, BA; Gangoiti, J; Nyhan, WL; Willis, M1
Boysen, G; Horvath, TD; Matthews, NI; Mock, DM; Stratton, SL1
Bogusiewicz, A; Boysen, G; Dawson, AM; Henrich, CL; Horvath, TD; Matthews, NI; Mock, DM; Moran, JH; Owen, SN; Spencer, HJ; Stratton, SL1
Caudill, MA; Gayle, A; Jiang, X; Lucas, LK; Malysheva, O; Perry, CA; West, AA; Yan, J1
Nakamura, T; Shibata, K; Tsuji, A1
Deng, Z; He, H; Hong, Q; Liu, S; Luo, X; Zhang, Y; Zheng, X1

Reviews

2 review(s) available for biotin and beta-hydroxyisovaleric acid

ArticleYear
Biotin status: which are valid indicators and how do we know?
    The Journal of nutrition, 1999, Volume: 129, Issue:2S Suppl

    Topics: Animals; Biotin; Female; Humans; Nutritional Status; Pregnancy; Valerates

1999
[Biotin (vitamin H)].
    Nihon rinsho. Japanese journal of clinical medicine, 2004, Volume: 62 Suppl 12

    Topics: Biological Assay; Biomarkers; Biotin; Biotinidase; Biotinidase Deficiency; Dermatitis, Atopic; Epilepsies, Partial; Humans; Reference Values; Specimen Handling; Valerates

2004

Trials

3 trial(s) available for biotin and beta-hydroxyisovaleric acid

ArticleYear
Marginal biotin deficiency during normal pregnancy.
    The American journal of clinical nutrition, 2002, Volume: 75, Issue:2

    Topics: Adult; Biotin; Deficiency Diseases; Female; Humans; Pregnancy; Pregnancy Complications; Valerates

2002
Lymphocyte propionyl-CoA carboxylase and its activation by biotin are sensitive indicators of marginal biotin deficiency in humans.
    The American journal of clinical nutrition, 2006, Volume: 84, Issue:2

    Topics: Adult; Analysis of Variance; Biomarkers; Biotin; Cross-Over Studies; Deficiency Diseases; Female; Humans; Lymphocytes; Male; Methylmalonyl-CoA Decarboxylase; Nutrition Assessment; Nutritional Status; Sensitivity and Specificity; Valerates

2006
Pregnancy and lactation alter biomarkers of biotin metabolism in women consuming a controlled diet.
    The Journal of nutrition, 2014, Volume: 144, Issue:12

    Topics: Adult; Biomarkers; Biotin; Carbon-Carbon Ligases; Carnitine; Choline; Chromatography, Liquid; Diet; Dietary Supplements; Female; Humans; Lactation; Leucine; Milk, Human; New York; Patient Compliance; Pregnancy; Tandem Mass Spectrometry; Valerates; Young Adult

2014

Other Studies

31 other study(ies) available for biotin and beta-hydroxyisovaleric acid

ArticleYear
Biotin deficiency in rats: disturbances of leucine metabolism are detectable early.
    The Journal of nutrition, 1992, Volume: 122, Issue:7

    Topics: Animals; Biotin; Gas Chromatography-Mass Spectrometry; Leucine; Male; Rats; Rats, Inbred Strains; Valerates

1992
Quantification of urinary 3-hydroxyisovaleric acid using deuterated 3-hydroxyisovaleric acid as internal standard.
    Biomedical & environmental mass spectrometry, 1989, Volume: 18, Issue:9

    Topics: Animals; Biotin; Deuterium; Gas Chromatography-Mass Spectrometry; Magnetic Resonance Spectroscopy; Rats; Reference Standards; Valerates

1989
Biotin-responsive multiple carboxylase deficiency in an 8-year-old boy with normal serum biotinidase and fibroblast holocarboxylase-synthetase activities.
    Journal of inherited metabolic disease, 1988, Volume: 11, Issue:3

    Topics: Amidohydrolases; Biotin; Biotinidase; Carbon-Nitrogen Ligases; Child; Fibroblasts; Glycine; Humans; Kinetics; Ligases; Male; Multiple Carboxylase Deficiency; Valerates

1988
Abnormal organic aciduria in biotin deficiency: the rat is similar to the human.
    The Journal of laboratory and clinical medicine, 1988, Volume: 112, Issue:2

    Topics: Animals; Biotin; Disease Models, Animal; Gas Chromatography-Mass Spectrometry; Glycine; Male; Random Allocation; Rats; Rats, Inbred Strains; Valerates

1988
Biotin deficiency complicating parenteral alimentation: diagnosis, metabolic repercussions, and treatment.
    The Journal of pediatrics, 1985, Volume: 106, Issue:5

    Topics: Alopecia; Biotin; Citrates; Diagnosis, Differential; Erythema; Fatty Acids, Essential; Female; Glycine; Humans; Infant; Isomerism; Lactates; Lactic Acid; Male; Nervous System Diseases; Parenteral Nutrition; Valerates; Zinc

1985
Organic aciduria in late-onset biotin-responsive multiple carboxylase deficiency.
    Journal of inherited metabolic disease, 1985, Volume: 8 Suppl 2

    Topics: Biotin; Carbon-Carbon Ligases; Carboxy-Lyases; Female; Humans; Infant; Lactates; Lactic Acid; Ligases; Methylmalonyl-CoA Decarboxylase; Pyruvate Carboxylase Deficiency Disease; Valerates

1985
A combined defect of three mitochondrial carboxylases presenting as biotin-responsive 3-methylcrotonyl glycinuria and 3-hydroxyisovaleric aciduria.
    Clinica chimica acta; international journal of clinical chemistry, 1980, Jan-15, Volume: 100, Issue:2

    Topics: Acidosis; Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Biotin; Butyrates; Carbon Dioxide; Carboxy-Lyases; Child, Preschool; Crotonates; Female; Fibroblasts; Glycine; Humans; Hydroxy Acids; Ligases; Mitochondria; Propionates; Pyruvate Carboxylase Deficiency Disease; Valerates

1980
Two forms of biotin-responsive multiple carboxylase deficiency.
    Journal of inherited metabolic disease, 1981, Volume: 4, Issue:2

    Topics: Acute Disease; Biotin; Carbon-Carbon Ligases; Chronic Disease; Fibroblasts; Humans; Hydroxy Acids; Infant; Lactates; Lactic Acid; Ligases; Lymphocytes; Pyruvate Carboxylase Deficiency Disease; Valerates

1981
Partial methylcrotonyl-coenzyme A carboxylase deficiency in an infant with failure to thrive, gastrointestinal dysfunction, and hypertonia.
    Pediatrics, 1993, Volume: 91, Issue:3

    Topics: Biotin; Carbon-Carbon Ligases; Failure to Thrive; Gastrointestinal Diseases; Glycine; Humans; Infant; Ligases; Male; Metabolism, Inborn Errors; Muscle Hypertonia; Valerates

1993
Isolated biotin-resistant 3-methylcrotonyl-CoA carboxylase deficiency: long-term outcome in a case with neonatal onset.
    European journal of pediatrics, 1996, Volume: 155, Issue:7

    Topics: Age of Onset; Biotin; Carbon-Carbon Ligases; Developmental Disabilities; Diet, Protein-Restricted; Epilepsy, Generalized; Humans; Infant, Newborn; Ligases; Male; Metabolism, Inborn Errors; Patient Compliance; Valerates

1996
Increased urinary excretion of 3-hydroxyisovaleric acid and decreased urinary excretion of biotin are sensitive early indicators of decreased biotin status in experimental biotin deficiency.
    The American journal of clinical nutrition, 1997, Volume: 65, Issue:4

    Topics: Adult; Analysis of Variance; Avidin; Biotin; Chromatography, High Pressure Liquid; Egg White; Female; Gas Chromatography-Mass Spectrometry; Humans; Male; Reference Values; Reproducibility of Results; Sensitivity and Specificity; Valerates

1997
Biotin status assessed longitudinally in pregnant women.
    The Journal of nutrition, 1997, Volume: 127, Issue:5

    Topics: Adult; Analysis of Variance; Biotin; Biotransformation; Chromatography, High Pressure Liquid; Female; Gas Chromatography-Mass Spectrometry; Humans; Longitudinal Studies; Pregnancy; Retrospective Studies; Valerates

1997
Conflicting indicators of biotin status from a cross-sectional study of normal pregnancy.
    Journal of the American College of Nutrition, 1997, Volume: 16, Issue:3

    Topics: Adult; Biotin; Case-Control Studies; Chromatography, High Pressure Liquid; Cross-Sectional Studies; Female; Gas Chromatography-Mass Spectrometry; Humans; Nutritional Status; Pregnancy; Reference Values; Valerates

1997
Biotin catabolism is accelerated in adults receiving long-term therapy with anticonvulsants.
    Neurology, 1997, Volume: 49, Issue:5

    Topics: Adolescent; Adult; Anticonvulsants; Biotin; Epilepsy; Female; Humans; Male; Middle Aged; Time Factors; Valerates

1997
Cerebral metabolic changes in biotinidase deficiency.
    Journal of inherited metabolic disease, 1997, Volume: 20, Issue:6

    Topics: Amidohydrolases; Biotin; Biotinidase; Brain; Electroencephalography; Humans; Infant; Lactic Acid; Magnetic Resonance Imaging; Male; Pyruvic Acid; Seizures; Valerates

1997
Disturbances in biotin metabolism in children undergoing long-term anticonvulsant therapy.
    Journal of pediatric gastroenterology and nutrition, 1998, Volume: 26, Issue:3

    Topics: Anticonvulsants; Biotin; Carbamazepine; Child; Female; Humans; Male; Phenobarbital; Phenytoin; Valerates

1998
Certain immune markers are not good indicators of mild to moderate biotin deficiency in rats.
    The Journal of nutrition, 2001, Volume: 131, Issue:12

    Topics: Animals; Biomarkers; Biotin; Carbon-Carbon Ligases; Cell Division; Cells, Cultured; Cytokines; Haemophilus Vaccines; Immunoglobulin G; Interferon-gamma; Interleukin-4; Killer Cells, Natural; Leukocytes, Mononuclear; Liver; Lymphocyte Activation; Lymphocyte Subsets; Lymphocytes; Male; Rats; Rats, Sprague-Dawley; Spleen; Thymus Gland; Valerates

2001
Biotin dependency due to a defect in biotin transport.
    The Journal of clinical investigation, 2002, Volume: 109, Issue:12

    Topics: Amidohydrolases; Biological Transport; Biotin; Biotinidase; Carbon-Carbon Ligases; Carboxy-Lyases; Carrier Proteins; Cell Line, Transformed; Child, Preschool; Female; Humans; Lactic Acid; Leukocytes, Mononuclear; Male; Membrane Glycoproteins; Methylmalonyl-CoA Decarboxylase; Pyruvate Carboxylase; Substance Withdrawal Syndrome; Symporters; Valerates

2002
Indicators of marginal biotin deficiency and repletion in humans: validation of 3-hydroxyisovaleric acid excretion and a leucine challenge.
    The American journal of clinical nutrition, 2002, Volume: 76, Issue:5

    Topics: Adult; Biotin; Diet; Dose-Response Relationship, Drug; Egg White; Female; Humans; Leucine; Male; Time Factors; Valerates

2002
Partial response to biotin therapy in a patient with holocarboxylase synthetase deficiency: clinical, biochemical, and molecular genetic aspects.
    Molecular genetics and metabolism, 2003, Volume: 79, Issue:3

    Topics: Age of Onset; Biotin; Carbon-Carbon Ligases; Carbon-Nitrogen Ligases; Child; DNA Mutational Analysis; Female; Genes, Recessive; Holocarboxylase Synthetase Deficiency; Humans; Methylmalonyl-CoA Decarboxylase; Mutation; Phenotype; Pyruvate Carboxylase; Reverse Transcriptase Polymerase Chain Reaction; RNA Splicing; Valerates

2003
Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy.
    American journal of human genetics, 2004, Volume: 75, Issue:5

    Topics: Alleles; Base Sequence; Biotin; Carbon-Carbon Ligases; DNA Mutational Analysis; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression; Genetic Vectors; Germany; Glycine; Greece; Humans; Infant, Newborn; Male; Mitochondrial Diseases; Molecular Sequence Data; Mutation; Reverse Transcriptase Polymerase Chain Reaction; Sequence Analysis, DNA; Transfection; Valerates

2004
Smoking accelerates biotin catabolism in women.
    The American journal of clinical nutrition, 2004, Volume: 80, Issue:4

    Topics: Adult; Avidin; Biomarkers; Biotin; Carbon-Carbon Ligases; Case-Control Studies; Chromatography, High Pressure Liquid; Cohort Studies; Creatinine; Female; Humans; Male; Middle Aged; Sex Factors; Smoking; Valerates

2004
Measurement of 3-hydroxyisovaleric acid in urine of biotin-deficient infants and mice by HPLC.
    The Journal of nutrition, 2005, Volume: 135, Issue:3

    Topics: Animals; Biomarkers; Biotin; Chromatography, High Pressure Liquid; Deficiency Diseases; Humans; Infant; Male; Mice; Mice, Inbred ICR; Regression Analysis; Valerates

2005
Changes in measures of biotin status do not reflect milk yield responses when dairy cows are fed supplemental biotin.
    Journal of dairy science, 2007, Volume: 90, Issue:3

    Topics: Animals; Biotin; Cattle; Dairying; Diet; Dietary Supplements; Fatty Acids, Volatile; Female; Lactation; Milk; Rumen; Time Factors; Valerates

2007
Biotin deficiency affects both synthesis and degradation of pyruvate carboxylase in rat primary hepatocyte cultures.
    Molecular genetics and metabolism, 2007, Volume: 92, Issue:3

    Topics: Animals; Biotin; Biotinylation; Hepatocytes; Lymphocytes; Male; Methylmalonyl-CoA Decarboxylase; Pyruvate Carboxylase; Rats; Rats, Wistar; Valerates

2007
Effects of biotin deficiency on embryonic development in mice.
    Nutrition (Burbank, Los Angeles County, Calif.), 2009, Volume: 25, Issue:1

    Topics: Animals; Biotin; Chromatography, High Pressure Liquid; Congenital Abnormalities; Embryonic Development; Energy Metabolism; Female; Fetus; Gestational Age; Male; Mice; Mice, Inbred ICR; Nutritional Requirements; Pregnancy; Pregnancy Outcome; Pregnancy, Animal; Random Allocation; Valerates

2009
Positive newborn screen in the biochemically normal infant of a mother with treated holocarboxylase synthetase deficiency.
    Journal of inherited metabolic disease, 2009, Volume: 32 Suppl 1

    Topics: Adult; Biotin; Carnitine; False Positive Reactions; Female; Holocarboxylase Synthetase Deficiency; Humans; Infant, Newborn; Male; Maternal-Fetal Exchange; Neonatal Screening; Pregnancy; Pregnancy Complications; Valerates

2009
Measurement of 3-hydroxyisovaleric acid in urine from marginally biotin-deficient humans by UPLC-MS/MS.
    Analytical and bioanalytical chemistry, 2011, Volume: 401, Issue:9

    Topics: Adult; Biomarkers; Biotin; Biotinidase Deficiency; Calibration; Chromatography, Liquid; Creatinine; Egg White; Female; Humans; Male; Reference Standards; Reproducibility of Results; Sensitivity and Specificity; Tandem Mass Spectrometry; Valerates

2011
Urinary excretion of 3-hydroxyisovaleric acid and 3-hydroxyisovaleryl carnitine increases in response to a leucine challenge in marginally biotin-deficient humans.
    The Journal of nutrition, 2011, Volume: 141, Issue:11

    Topics: Adult; Biotin; Carnitine; Cohort Studies; Female; Humans; Leucine; Male; Valerates

2011
Biotin-deficient diet induces chromosome misalignment and spindle defects in mouse oocytes.
    Bioscience, biotechnology, and biochemistry, 2015, Volume: 79, Issue:2

    Topics: Animals; Biotin; Body Weight; Cell Count; Chromosome Aberrations; Diet; Eating; Estradiol; Estrous Cycle; Female; Liver; Mice; Mice, Inbred ICR; Oocytes; Organ Size; Ovary; Spindle Apparatus; Uterus; Valerates

2015
Delayed Biotin Therapy in a Child with Atypical Profound Biotinidase Deficiency: Late Arrival of the Truth and a Lesson Worth Thinking.
    International journal of molecular sciences, 2023, Jun-16, Volume: 24, Issue:12

    Topics: Biotin; Biotinidase; Biotinidase Deficiency; Humans; Valerates

2023