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         Tyrosinemia:     more detail
  1. Tyrosinemia
  2. Palmoplantar Keratodermas: Papillon-lefèvre Syndrome, Palmoplantar Keratoderma, Meleda Disease, Tyrosinemia Type Ii, Howel-evans Syndrome
  3. Tyrosinemia - A Bibliography and Dictionary for Physicians, Patients, and Genome Researchers by Philip M. Parker, 2007-07-19
  4. Tyrosinemia: Webster's Timeline History, 1967 - 2007 by Icon Group International, 2010-06-11
  5. Dietary management of inherited metabolic disease: Phenylketonuria, galactosemia, tyrosinemia, homocystinuria, maple syrup urine disease by Phyllis B Acosta, 1976

81. Health Library -
Provides support and information for parents of children with tyrosinemia typeI (aka alpha foetoprotein or hepatorenal type hereditary tyrosinemia).
http://yalenewhavenhealth.org/library/healthguide/en-us/SelfHelp/topic.asp?hwid=

82. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Rare_Disorders/Tyr
National Library of Medicine The synonyms of tyrosinemia 11, tyrosinemia -For all people interested in connecting with others with this disease.
http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Rare_Disord
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83. THE MERCK MANUAL--SECOND HOME EDITION, Amino Acid Metabolism In Ch. 282, Heredit
Children with tyrosinemia are unable to completely metabolize the amino acid tyrosine Unlike type I tyrosinemia, restriction of tyrosine in the diet can
http://www.merck.com/mmhe/sec23/ch282/ch282c.html
var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Children's Health Issues Chapter Hereditary Metabolic Disorders Topics Introduction Amino Acid Metabolism Carbohydrate Metabolism Lipid Metabolism Pyruvate Metabolism Amino Acid Metabolism Buy The Book Print This Topic Email This Topic Pronunciations amniocentesis chorionic villus sampling cobalamin cystinuria ... pyruvate metabolism Amino acids are the building blocks of proteins and have many functions in the body. Hereditary disorders of amino acid processing can be the result of defects either in the breakdown of amino acids or in the body's ability to get the amino acids into cells. Because these disorders produce symptoms early in life, newborns are routinely screened for several common ones. In the United States, newborns are commonly screened for phenylketonuria, maple syrup urine disease, homocystinuria, tyrosinemia, and a number of other inherited disorders, although screening varies from state to state.

84. Tyrosinemia Links
This tyrosinemia links page contains links to support groups, education sites,and metabolic programs.
http://www.formydiet.com/Information/LinkList.aspx?c=5

85. IngentaConnect In Vivo Correction Of Murine Tyrosinemia Type I By DNA-Mediated T
In Vivo Correction of Murine tyrosinemia Type I by DNAMediated Transposition.Authors Montini E. 1; Held PK 1; Noll M. 1; Morcinek N. 1; Al-Dhalimy M. 1;
http://www.ingentaconnect.com/content/ap/ot/2002/00000006/00000006/art00812

86. Mouse Models For The Human Disease Of Chronic Hereditary Tyrosinemia
When a section of mouse chromosome 7 containing the coat color c gene is deletedby exposing mice to radiation, albino mice are born with a white,
http://www.eurekalert.org/features/doe/2001-06/drnl-mmf061802.php

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Mouse models for the human disease of chronic hereditary tyrosinemia
ORNL researchers found that a nearby gene that is also knocked out by irradiation causes chronic hereditary tyrosinemia in mice, a disease that also afflicts humans. Patients with this disease can develop a wide range of liver and kidney problems, as well as problems affecting the eye, the skin of the feet and hands, and the central nervous system. Using the powerful mutagen ENU to alter a single DNA base pair in this gene, ORNL biologists recently produced two mouse models that more closely mimic the gene mutation that causes chronic hereditary tyrosinemia in humans. Normal mice and people metabolize tyrosine, an amino acid available in food, to make melanin, a type of pigment produced in large amounts by dark-skinned people. But people and mice with the disease lack a normally functioning protein (enzyme) to carry out one step of the tyrosine metabolism process, which involves a series of enzymes. So, unless people with this disease are put on a special tyrosine-free diet, a substance that is not broken down because of the absence of a normally functioning enzyme will build up to toxic levels in the liver and kidneys, a fatal condition in mice. By sequencing the same chromosomal region from both normal and abnormal mice and comparing the sequences, ORNL biologists identified new mouse models that carry mutations in this enzyme in the tyrosine breakdown pathway. "Mice with this disease die of poisoned livers," says ORNL biologist Dabney Johnson. "A by-product of the botched metabolism process is succinyl acetone, which accumulates in the liver and is excreted in urine where it serves as a diagnostic indicator of the disease.

87. Tyrosinemia - Definition Of Tyrosinemia In The Medical Dictionary - By The Free
Definition of tyrosinemia in the Medical Dictionary and Thesaurus. tyrosinemiaexplanation. Information about tyrosinemia in Free online English dictionary.
http://medical-dictionary.thefreedictionary.com/tyrosinemia
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Cite / link Email Feedback ty·ro·si·ne·mi·a (t r -s -n m n. An inherited disorder of tyrosine metabolism marked by an increase in the concentration of tyrosine in the blood, an increase in urinary excretion of tyrosine and related compounds, hepatosplenomegaly, nodular cirrhosis of the liver, multiple renal tubular reabsorptive defects, and vitamin D-resistant rickets. Mentioned in No references found Medical browser Full browser typing Tyr tyramine tyrocidine ... tyrosine tyrosinemia tyrosinosis tyrosinuria tyrosyluria tyrothricin ... Tyrosine kinases tyrosinemia tyrosinosis tyrosinuria tyrosyluria tyrothricin ... Tyrrell County, NC

88. Arch Pediatr Adolesc Med -- Abstract: Dietary Management Of Oculocutaneous Tyros
Dietary management of oculocutaneous tyrosinemia in an 11year-old child An 11-year-old girl with keratitis and plantar keratosis had tyrosinemia.
http://archpedi.ama-assn.org/cgi/content/abstract/137/10/995
Select Journal or Resource JAMA Archives of Dermatology Facial Plastic Surgery Family Medicine (1992-2000) General Psychiatry Internal Medicine Neurology Ophthalmology Surgery Student JAMA (1998-2004) JAMA CareerNet For The Media Meetings Peer Review Congress
Vol. 137 No. 10, October 1983 Featured Link E-mail Alerts ARTICLE Article Options Send to a Friend Readers Reply Submit a reply Similar articles in this journal Literature Track Add to File Drawer Download to Citation Manager Articles in PubMed by Ney D Nyhan WL Contact me when this article is cited
Dietary management of oculocutaneous tyrosinemia in an 11-year-old child
D. Ney, C. Bay, J. A. Schneider, D. Kelts and W. L. Nyhan
An 11-year-old girl with keratitis and plantar keratosis had tyrosinemia. The concentration of tyrosine in the plasma was 16.5 mg/dL. Dietary intake of phenylalanine and tyrosine was systematically varied, and the plasma concentrations of tyrosine and nitrogen balance were studied. It was necessary to achieve a total intake of phenylalanine and tyrosine less than 100 mg/kg/day to obtain plasma concentrations of tyrosine of less than 10 mg/dL. After dietary therapy was started, the keratitis resolved promptly

89. AnyWho: Internet Directory Assistance; Yellow Pages, White Pages, Toll-Free Numb
Chapitre 3 V259L, associated with atypical symptoms of hereditary Translate this page A new mutation of the fah gene, responsible for hereditary tyrosinemia type I, Hereditary tyrosinemia type I is the most severe disease of the tyrosine
http://www.anywho.com/cgi-bin/webdrill?catkey=gwd/Top/Health/Conditions_and_Dise

90. Vindex - Zoekresultaten Voor
The synonyms of tyrosinemia 11, a summary and major features. URLhttp//www.nlm.nih.gov/cgi/jablonski/syndrome_cgi?term=tyrosinemia+II field=name
http://www.vindex.nl/dmoz.jspx?topic_id=Top/Health/Conditions_and_Diseases/Rare_

91. Atlas Tyrosinemia Tyrosinemie
Translate this page ATLAS DE PEDIATRIE. Tyrosinémie/tyrosinemia. INDEX. Tyrosinémie tyrosinemia.Photophobie - kératite dans la tyrosinémie taux excessifs de tyrosine
http://www.icampus.ucl.ac.be/PEDIHEPA/document/pedihepa/atlas3.htm

92. On The Enzymic Defects In Hereditary Tyrosinemia -- Lindblad Et Al. 74 (10): 464
Urine from patients with hereditary tyrosinemia contained an inhibitor that wasisolated and Hepatocyte injury in tyrosinemia type 1 is induced by
http://www.pnas.org/cgi/content/abstract/74/10/4641
This Article Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted ... Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to My File Cabinet ... Cited by other online articles PubMed PubMed Citation Articles by Lindblad, B. Articles by Steen, G. October 1, 1977
On the Enzymic Defects in Hereditary Tyrosinemia Bengt Lindblad, Sven Lindstedt, and Goran Steen
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C. E.L. Lim, K. I. Matthaei, A. C. Blackburn, R. P. Davis, J. E. Dahlstrom, M. E. Koina, M.W. Anders, and P. G. Board
Mice Deficient in Glutathione Transferase Zeta/Maleylacetoacetate Isomerase Exhibit a Range of Pathological Changes and Elevated Expression of Alpha, Mu, and Pi Class Glutathione Transferases
Am. J. Pathol., August 1, 2004; 165(2): 679 - 693.
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Plant Physiology, April 1, 2004; 134(4): 1388 - 1400.

93. Health Library -
next . Children s Liver Alliance 3835 Richmond Avenue, Box 190
http://12.42.224.150/library/healthguide/en-us/SelfHelp/topic.asp?hwid=shc29tyr

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