John Menkes
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Most Influential Person Now
American author
Why Is John Menkes Influential?
(Suggest an Edit or Addition)According to Wikipedia, John Hans Menkes was an Austrian-American pediatric neurologist and author of fictional novels and plays. He identified two inherited diseases: maple syrup urine disease which is a defect in amino acid metabolism, and a defect in copper transport which bears his name. In addition to a career in academic medicine, he pursued a career in writing, publishing novels and plays.
John Menkes's Published Works
Number of citations in a given year to any of this author's works
Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author
Published Works
- A sex-linked recessive disorder with retardation of growth, peculiar hair, and focal cerebral and cerebellar degeneration. (1962) (861)
- A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance. (1954) (370)
- Textbook of child neurology (1980) (236)
- A twenty-five year follow-up study on the hyperkinetic child with minimal brain dysfunction. (1967) (187)
- Cerebrotendinous xanthomatosis. The storage of cholestanol within the nervous system. (1968) (147)
- Maple syrup disease; isolation and identification of organic acids in the urine. (1959) (134)
- Generalized convulsive seizure as an adverse event following immunization: case definition and guidelines for data collection, analysis, and presentation. (2004) (102)
- Relationship of elevated blood tyrosine to the ultimate intellectual performance of premature infants. (1972) (95)
- Biosynthesis of long chain fatty acids by subcellular particles of mature brain. (1968) (91)
- Kinky hair disease: Twenty five years later (1988) (88)
- HEREDITARY PARTIAL AGENESIS OF CORPUS CALLOSUM; BIOCHEMICAL AND PATHOLOGICAL STUDIES. (1964) (79)
- Growth and Development of the Thyroidectomized Ovine Fetus (1974) (79)
- Transient tyrosinemia of the newborn: dietary and clinical aspects. (1967) (78)
- Kinky hair disease. (1972) (77)
- Adrenoleukodystrophy: Accumulation of cholesterol esters with very long chain fatty acids (1977) (76)
- The pathogenesis of mental retardation in phenylketonuria and other inborn errors of amino acid metabolism. (1967) (69)
- Letter: Toxic polyneuropathy due to methyl n-butyl ketone. (1976) (67)
- Concentration and fatty acid composition of cerebrosides and sulfatides in mature and immature human brain. (1966) (67)
- Clinical and MR correlates in children with extrapyramidal cerebral palsy. (1994) (66)
- Niemann-Pick disease. Morphologic and biochemical studies in the visceral form with late central nervous system involvement (Crocker's group C). (1969) (63)
- Globoid cell leukodystrophy (Krabbe's disease) (1971) (59)
- Early Feeding History of Children with Learning Disorders (1977) (54)
- Encephalitis lethargica‐like illness in a girl with mycoplasma infection (1988) (53)
- A New Anticonvulsant in the Management of Minor Motor Seizures (1970) (52)
- The causes for low spinal fluid sugar in bacterial meningitis: another look. (1969) (47)
- THE CULTURE OF DISSOCIATED CELLS FROM RAT CEREBRAL CORTEX (1973) (44)
- CEREBRAL LIPIDS IN MAPLE SYRUP DISEASE. (1965) (44)
- POLYUNSATURATED FATTY ACID METABOLISM IN NEUROBLASTOMA CELLS IN CULTURE 1 (1975) (44)
- Idiopathic hyperglycinemia: isolation and identification of three previously undescribed urinary ketones. (1966) (44)
- Fibroblast cultures in Huntington's disease. (1973) (43)
- Menkes disease and Wilson disease: two sides of the same copper coin. Part II: Wilson disease. (1999) (43)
- Cerebral proteolipids in phenylketonuria (1968) (43)
- Regional distribution of messenger RNAs in postmortem human brain (1986) (40)
- Fatty Acid Synthesis in the Developing Brain (1969) (39)
- Juvenile GM2 gangliosidosis. Biochemical and ultrastructural studies on a new variant of Tay-Sachs disease. (1971) (38)
- Menkes disease and Wilson disease: two sides of the same copper coin. Part I: Menkes disease. (1999) (38)
- Abnormalities in growth of skin fibroblasts of patients with Huntington's disease (1977) (38)
- Diffuse sclerosis and Addison's disease: biochemical studies on gray matter, white matter, and myelin. (1973) (37)
- ISOLATION AND CHARACTERIZATION OF THE MAIN SPLENIC GLYCOLIPIDS IN THE NORMAL ORGAN AND IN GAUCHER'S DISEASE: EVIDENCE FOR THE SITE OF METABOLIC BLOCK (1965) (36)
- See‐saw winking in a familial oral‐facial‐digital syndrome (1971) (35)
- Cerebral lipids in phenylketonuria. (1966) (33)
- Unusual Dyskinesia Complicating Cardiopulmonary Bypass Surgery (1993) (33)
- Maple syrup disease. Effects of dietary therapy on cerebral lipids. (1967) (32)
- Hypotonic-Hyporesponsive Episode (HHE) as an adverse event following immunization: case definition and guidelines for data collection, analysis, and presentation. (2004) (31)
- Effect of elevated blood tyrosine on subsequent intellectual development of premature infants. (1965) (30)
- Isolation and characterization of the main splenic glycolipids in Gaucer's disease: Evidence for the site of metabolic block (1964) (30)
- Developmental retardation associated with an abnormality in tyrosine metabolism. Report of a case. (1961) (29)
- Maple syrup disease (1959) (29)
- Fluoranthene Derivatives. III. 2-Nitrofluoranthene and 2-Aminofluoranthene1 (1956) (29)
- Workshop on neurologic complications of pertussis and pertussis vaccination. (1990) (28)
- Molecular composition of the major glycolipids in globoid cell leukodystrophy (1966) (28)
- GLYCERIDE METABOLISM IN CULTURED CELLS DISSOCIATED FROM RAT CEREBRAL CORTEX 1 (1973) (28)
- Ultrastructure of experimentally produced globoid cells in the rat. (1970) (27)
- Maternal phenylketonuria. The composition of cerebral lipids in an affected offspring. (1969) (26)
- Association of autism, retinoblastoma, and reduced esterase D activity. (1988) (25)
- Huntington's disease: delayed hypersensitivity in vitro to human central nervous system antigens. (1977) (25)
- Energy Metabolism of Brain in Human Protein-Calorie Malnutrition (1977) (24)
- Late infantile neuro visceral storage disease with curvilinear bodies (1971) (24)
- How to Construct a Neural Tube (2000) (22)
- Changes in the adenosine triphosphate and creatine phosphate content of the rabbit uterus throughout sexual maturation and after ovulation. (1952) (21)
- Neonatal hyperphenylalaninemia: a differential diagnosis. (1970) (20)
- Bilirubin Interaction with Ganglioside: Possible Mechanism in Kernicterus (1975) (20)
- The Pattern of Urinary Alpha Keto Acids in Various Neurological Diseases (1960) (20)
- Detection of point mutations associated with genetic diseases by an exon scanning technique. (1990) (20)
- Lipofuscin (ceroid) storage disease of the brain neuropathological and neurochemical studies. (1970) (19)
- Menkes disease. New ocular and electroretinographic findings. (1998) (19)
- Cerebral palsy due to chromosomal anomalies and continuous gene syndromes. (2006) (19)
- Huntington's disease—growth of fibroblast cultures in lipid‐deficient medium: A preliminary report (1977) (18)
- The cerebroretinal degenerations. (1971) (16)
- ISOLATION AND CHARACTERIZATION OF THE PRINCIPAL CEREBRAL GLYCOLIPIDS IN THE INFANTILE AND ADULT FORMS OF GAUCHER'S DISEASE* (1967) (15)
- Cellular immune responses in Huntington's disease (H.D.). Detection of H.D. and multiple sclerosis (M.S.) brain antigenicity by H.D. but not M.S. lymphocytes. (1977) (15)
- Normal glucose, insulin, and growth hormone responses to oral glucose in Huntington's disease (1974) (15)
- LEAD AND NEUROBEHAVIOURAL DEFICIT IN CHILDREN (1979) (15)
- [Globoid cell leukodystrophy]. (1970) (15)
- LIPID METABOLISM IN BRAIN TISSUE EXPLANTS 1 (1971) (14)
- Cystathioninuria accompanying necrotizing encephalomyelopathy of childhood. (1968) (14)
- Chemical studies of two cerebral biopsies in juvenile metachromatic leukodystrophy: the molecular composition of cerebrosides and sulfatides. (1966) (14)
- Cellular immune responses in Huntington disease Specificity of brain antigenicity detected with Huntington disease lymphocytes (1978) (14)
- Adverse events following immunization: assessing probability of causation. (1989) (13)
- Effect of essential fatty acids on proliferation of two neural tumor lines. (1975) (12)
- THE METABOLISM OF PHENYLALANINE AND TYROSINE IN THE PREMATURE INFANT. (1963) (12)
- Genetic disorders of mitochondrial function. (1987) (12)
- A NEUROBEHAVIORAL SYNDROME AFTER FAILURE TO THRIVE ON CHLORIDE‐DEFICIENT FORMULA (1991) (11)
- Sections on Child Neurology and Neurochemistry (Joint Session) (1973) (11)
- Brain Damage in the Aminoacidurias (1972) (11)
- Subdural haematoma, non-accidental head injury or ...? (2001) (10)
- IMPROVING THE LONG-TERM OUTLOOK IN BACTERIAL MENINGITIS (1979) (10)
- Huntington disease: finding the gene and after. (1988) (10)
- Cerebral Lipidosis of Unclear Nature (1962) (10)
- On failing in school. (1976) (9)
- Neurologic disorders of gastroesophageal function. (1988) (9)
- Polyenoic acid metabolism in cultured human skin fibroblasts (1978) (9)
- Catecholaminergic activity in idiopathic torsion dystonia (1987) (8)
- Tay-sachs disease. Demonstration of the stored ganglioside in cultured cells from brain biopsy. (1969) (8)
- HEREDITARY PARTIAL AGENESIS OF THE CORPUS CALLOSUM. BIOCHEMICAL AND HISTOLOGICAL STUDIES. (1963) (8)
- Program for the detection of metabolic diseases. Chromatographic screening to find metabolic diseases, especially those involving the nervous system. (1962) (8)
- PHENYLKETONURIA OCCURRING IN AN AMERICAN NEGRO. (1964) (7)
- Pertussis Vaccine Encephalopathy (1990) (7)
- A new role for the school physician. (1972) (7)
- Effect of Thyroid on Fatty Acid Biosynthesis in Brain (1971) (7)
- Occult recurrence of nasopharyngeal rhabdomyosarcoma manifesting as isolated increased intracranial pressure. (1975) (7)
- Phenylalaninemia or classical phenylketonuria. (1970) (7)
- Biochemical and genetic aspects of mental retardation. (1966) (7)
- Cerebral Lipids in Down's Syndrome (1969) (6)
- Diagnosis and treatment of minor motor seizures. (1976) (6)
- To tap or not to tap. (1973) (6)
- Cholestanol storage in the nervous system of two patients with cerebrotendinous xanthomatosis. (1968) (6)
- Defects in purine metabolism and neurologic disease. (1968) (5)
- Treatment of Maple Syrup Disease (1959) (5)
- Polyenoic acid metabolism in cultured dissociated brain cells (2002) (5)
- The effect of branched‐chain alphaketo acids on the utilization of other branched‐chain compounds by lactobacillus casei (1962) (4)
- Metabolic errors affecting the nervous system. (1966) (4)
- Effect of Temperature on Fatty Acid Metabolism in Dissociated Cell Cultures of Developing Brain (1974) (4)
- A Didactic Autobiography (2001) (4)
- Incorporation and metabolism of fatty acids by cultured dissociated cells from rat cerebrum (1974) (4)
- Corkscrew basilar artery as an incidental finding on neuroimaging. (2007) (3)
- Huntington's disease: growth and fatty acid metabolism of fibroblast cultures in lipid-deficient medium. (1976) (3)
- A Clinical Guide to Inherited Metabolic Diseases (2003) (3)
- Biochemical methods in the detection and diagnosis of metabolic diseases affecting the nervous system. (1965) (3)
- Progressive non-conformism in the lateral geniculate nucleus of man; a problem in competence. (1958) (3)
- The leukodystrophies. (1990) (3)
- The history of hypertyrosinemia caused by high protein diets. (2006) (2)
- Biochemical Studies on Brain Explants and Fibroblast Cultures in Batten’s Disease (1972) (2)
- Normalcy and the gamma efferent system. (1960) (2)
- Slow viruses and inborn metabolic errors. Summary of a workshop sponsored by the National Institutes of Health. (1969) (2)
- Side-Reactions during Hydrogenolysis of 2,4-Dinitrophenylhydrazones of Aromatic Keto-Acids (1961) (2)
- MAPLE SYRUP DISEASE AND OTHER RARE DISORDERS OF AMINO ACID METABOLISM. (1964) (2)
- CHAPTER 108 – WILSON DISEASE (2007) (2)
- Chapter 31 – Wilson Disease (2003) (1)
- KINKY HAIR DISEASE (1972) (1)
- Lipids and Lipidoses. (1968) (1)
- A new anticonvulsant in the management of minor motor seizures. (1970) (1)
- Effect of elevated blood tyrosine on intellectual development of premature infants (1971) (1)
- Inherited Metabolic Diseases By Georg F Hoffmann, William L Nyhan, Johannes Zschocke, Stephen G Kahler, Ertan Mayatepek Lippincott Williams & Wilkins, Philadelphia 2002, 435pp, £29.95 ISBN: 0-7817-2900-9 (2002) (1)
- Low spinal fluid sugar in meningitis. (1970) (1)
- Prospects for biochemical treatment of genetic disorders. (1971) (1)
- Marvin Weil, California Pediatric Neurology Pioneer (2007) (1)
- Biology of Brain Dysfunction, Vol. I, edited by Gerald E. Gaull. New York/London: Plenum Press, 1973, 403 pp., $35.00 (1974) (1)
- Lipid metabolism of brain tissue in culture (1972) (1)
- Menkes disease: Authors’ reply (1999) (1)
- Tryptophan Metabolism in Psychotic Children (1967) (1)
- [DIFFUSE PULMONARY INTERSTITIAL FIBROSIS AND SCLERODERMA]. (1964) (1)
- Disorders of amino acid metabolism--1971. (1971) (1)
- Cerebral fatty acid biosynthesis in patients with leuko- and poliodystrophies. (1969) (1)
- In Reply: To Tap, or Not to Tap, in the Febrile Convulsion (1973) (1)
- Management of Status Epilepticus (1980) (1)
- Hyperglycinemia and methylmalonic aciduria. (1968) (1)
- The clinical evaluation of school difficulties. (1974) (1)
- Strategies for evaluating the child with suspected neurodegenerative disease. (1989) (1)
- Viral neurologic infections in children. (1977) (1)
- Lysosomal Disorders of the Brain, F.M. Platt, S.U. Walkley (Eds.). Oxford University Press (2004), hardback, 447 pages, £85.00, ISBN: 0-19-850878-6 (2005) (0)
- Huntington's disease masquerading as chronic schizophrenia. (1973) (0)
- Definition of perinatal asphyxia. (1989) (0)
- Spectrum of amentia. (1979) (0)
- Letter To The Editor (1970) (0)
- Lettrt To The Editor (1975) (0)
- Adrenoleukodystrophy (1977) (0)
- Biochemical approaches to the nosology of nervous system defects, III. (1971) (0)
- Searching the cause for seizures. (1974) (0)
- The Effect of Thyroxin on Fatty Acid Biosynthesis in Brain (1970) (0)
- THE DETECTION OF METABOLIC DISEASES OF THE NERVOUS SYSTEM. (1964) (0)
- [Medical treatment of rheumatoid arthritis]. (1972) (0)
- Paediatric Neurology (1980) (0)
- Torsion dystonis (1988) (0)
- Nutrition and the Developing Nervous System (1976) (0)
- Neurology of Hereditary Molecular and Metabolic Diseases of Children, 3rd rev. ed, Gilles Lyon, Edwin H. Kolodny, Gregory M. Pastores. McGraw-Hill Education, New York (2006), Hardcover, 500pp., £99.00, ISBN: 0071445080 (2007) (0)
- Letter to the Editor (1974) (0)
- Lipid metabolism of brain tissue in culture. In: lipids, malnutrition & the developing brain. (1971) (0)
- PERTUSSIS VACCINE ENCEPHALOTPATHY. AUTOR'S REPLY (1990) (0)
- Ramblings of a Peripatetic Paediatrician, Victor Dubowitz. The Memoir Club (2005), Hardback, 486pp., £20.00, ISBN: 1-84104-115-7 (2007) (0)
- Letter: Committee on Drugs attacked. (1974) (0)
- Letters to the Editor (1967) (0)
- Uncommon Causes of Stroke: Menkes disease (kinky hair disease) (2008) (0)
- CHANGES IN HUMAN BRAIN CEREBROSIDE STRUCTURE WITH MATURATION (1966) (0)
- Neurological Complications of Perinatal Asphyxia (2007) (0)
- Maple syrup disease and other disorders of keto acid metabolism. (1962) (0)
- VALPROATE IN THE U.S.A. (1976) (0)
- Medical Progress Disorders of Amino Acid Metabolism 1971 (2006) (0)
- Introduction: the treatment of paroxysmal disorders. (1974) (0)
- Atlas of Metabolic Diseases, W.L. Nyhan, B.A. Barshop, P.T. Ozand. A Hodder Arnold Publication, London (2005), 736pp., UK£150.00, hardback, ISBN: 0340809701 (2007) (0)
- [Classification of rheumatic diseases]. (1972) (0)
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