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         Mannosidosis:     more detail
  1. Mannosidosis: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Linnea, MS Wahl, 2005

21. CCHS Clinical Digital Library
International Society for mannosidosis Related Diseases Homepage. Alpha mannosidosis Table of contents. Canadian MPS Society Homepage
http://cchs-dl.slis.ua.edu/patientinfo/metabolism/inborn/lysosomalstorage/glycop
Patient/Family Resources by Topic: Metabolic Disorders
Alpha Mannosidosis Patient/Family Resources
Miscellaneous See also:

22. Mannosidosis
mannosidosis is a genetic disorder characterized by a lysosomal enzyme deficiency resulting in progressive mental and physical deterioration. A
http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

23. Alpha-mannosidosis: Information From Answers.com
Alphamannosidosis a-mannosidosis is a genetic disorder that causes progressive mental and physical deterioration.
http://www.answers.com/topic/alpha-mannosidosis
showHide_TellMeAbout2('false'); Business Entertainment Games Health ... More... On this page: Wikipedia Mentioned In Or search: - The Web - Images - News - Blogs - Shopping Alpha-mannosidosis Wikipedia Alpha-mannosidosis is a genetic disorder that causes progressive mental and physical deterioration. A defective enzyme , which normally helps to break down complex sugars in the lysosome , causes sugar build up and impair cell function.
This entry is from Wikipedia, the leading user-contributed encyclopedia. It may not have been reviewed by professional editors (see Mentioned In Alpha-mannosidosis is mentioned in the following topics: List of diseases starting with A Wikipedia information about Alpha-mannosidosis This article is licensed under the GNU Free Documentation License . It uses material from the Wikipedia article "Alpha-mannosidosis" More from Wikipedia Your Ad Here Jump to: Wikipedia Mentioned In Or search: - The Web - Images - News - Blogs - Shopping Send this page Print this page Link to this page Tell me about: Home About Tell a Friend Buzz ... Site Map

24. Mannosidosis: Definition And Much More From Answers.com
www.hgmp.mrc.ac.uk/dhmhdbin/hum-look-up?1051 Alpha-mannosidosis - Wikipedia, the free encyclopediaa-mannosidosis is a genetic disorder that causes progressive mental and physical Retrieved from http//en.wikipedia.org/wiki/Alpha-mannosidosis
http://www.answers.com/topic/mannosidosis
showHide_TellMeAbout2('false'); Business Entertainment Games Health ... More... On this page: Medical Mentioned In Or search: - The Web - Images - News - Blogs - Shopping mannosidosis Medical man·no·si·do·sis măn ə-sĭ-dō sĭs
n. An inherited disorder caused by the deficiency of an enzyme necessary for the metabolism of mannose and characterized by mental retardation, kyphosis, and an enlarged tongue, with the accumulation of mannose in the body tissues. Mentioned In mannosidosis is mentioned in the following topics: Alpha-mannosidosis List of diseases starting with B List of diseases starting with A List of diseases starting with M ... List of biomedical topics, M Medical definition of mannosidosis Houghton Mifflin Company More from Medical Your Ad Here Jump to: Medical Mentioned In Or search: - The Web - Images - News - Blogs - Shopping Send this page Print this page Link to this page Tell me about: Home About Tell a Friend Buzz ... Site Map

25. Mannosidosis
Alphamannosidosis only. Recurrent respiratory tract infections Malm, Dag, Ph.D; Nilssen, Oivind, Ph.D. Alpha-mannosidosis. www.genetests.org.
http://www.lysosomallearning.com/healthcare/about/lsd_hc_abt_mannosidosis.asp
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Genzyme Corporate
Search Contact Us Genzyme Websites Lyso Learning All Genzyme sites About Lysosomal Storage Disorders Disease Classification Disease Management var pageTitle="Mannosidosis"; var teaserText = "Exists in two forms, alpha and beta, which are found in separate gene locations but have similar effects."; Mannosidosis
Metabolic defect: alpha-mannosidosase deficiency Other glycan degradation diseases: aspartylglucosaminuria type I type II type I ... type II
Current page: Mannosidosis
Select Another Disease Select disease Acid sphingomyel. def. Aspartylglycosaminuria Cystinosis Fabry disease Farber disease Fucosidosis type I Fucosidosis type II Galactosialidosis Gaucher type I Gaucher type II Gaucher type III GM1 gangliosidosis I GM1 gangliosidosis II GM1 gangliosidosis III Infantile sialic acid Krabbé disease Mannosidosis Metachr. leukodys. I

26. Mannosidosis - Contents - Orthopaedic Rare Conditions Internet Database
Orthopaedic mailing lists, orthopaedic surgery education forums, comprehensive orthopaedic directory guide, educational resources for patients, surgeons,
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Dev. Team Editorial Board ISOST Global Consortium ... Orthogate Orthopaedic Rare Conditions Database (ORCID) Mannosidosis - Contents Cases Comments Bibliography
Mannosidosis
Glycogen Storage Inherited disorder with skeletal and other effects. Best account on the Internet ? Cases Comments Bibliography Official ONIM names and synonyms 248500 MANNOSIDOSIS , ALPHA B, LYSOSOMAL Alternative titles; symbols ALPHA-MANNOSIDOSIS
LYSOSOMAL ALPHA-D-MANNOSIDASE DEFICIENCY
ALPHA-MANNOSIDASE B DEFICIENCY
MANNOSIDASE, ALPHA B, LYSOSOMAL, INCLUDED; MANB, INCLUDED 248510 MANNOSIDOSIS , BETA; MANB1 Alternative titles; symbols BETA-MANNOSIDASE DEFICIENCY
MANNOSIDASE, BETA A, LYSOSOMAL, INCLUDED; MANBA, INCLUDED
MANNOSIDASE, BETA B, SOLUBLE, INCLUDED; MANBB, INCLUDED
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27. Mannosidosis - Bibliography - Orthopaedic Rare Conditions Internet Database
Orthopaedic mailing lists, orthopaedic surgery education forums, comprehensive orthopaedic directory guide, educational resources for patients, surgeons,
http://www.orthogate.com/orcid/mannobib.htm
Main Menu
ORCID Home ORCID Cases Submit ORCID Case ORCID Presentation ... Contact ORCID Editor
Sponsored Listings
About Orthogate
Dev. Team Editorial Board ISOST Global Consortium ... Orthogate Orthopaedic Rare Conditions Database (ORCID) Mannosidosis - Contents Cases Comments Bibliography
Mannosidosis
Bibliography
Internet Links OMIM Mannosidosis A Online Mendelian Inheritance in Man database OMIM Mannosidosis B Support group with some clinical information NORD Mannosidosis Page (National Organization for Rare Disorders) Mannosidosis, the Web Site PubMed Citations with special reference to Orthopaedics
Eckhoff DG, et al. [See Related Articles]
Severe destructive polyarthropathy in association with a metabolic storage disease. A case report.
J Bone Joint Surg Am. 1992 Sep;74(8):1257-61. Wall DA, et al. [See Related Articles]
Bone marrow transplantation for the treatment of alpha-mannosidosis.
J Pediatr. 1998 Aug;133(2):282-5.

28. Short Description Of Cell Lines. Pathology: Mannosidosis, Alpha B, Lysosomal *24
Pathology mannosidosis, alpha b, lysosomal *248500 OMIM record. By selecting the cell line name, you will receive the detailed description of the cell
http://www.biotech.ist.unige.it/cldb/pat111.html
Version
Short description of cell lines.
Pathology: mannosidosis, alpha b, lysosomal
OMIM record
By selecting the cell line name , you will receive the detailed description of the cell line
By selecting one of the terms between parentheses, you will receive the list of all relevant cell lines
You can search any term of the list by using the 'Find' utility of your browser
human, Caucasian
amnion GEIMM
human, Caucasian
...
By Beatrice...

29. Alpha-Mannosidosis
Alphamannosidosis is one of a group of very rare inherited disorders known as glycoprotein and related storage diseases. These disorders are caused by a
http://www.bchealthguide.org/kbase/nord/nord420.htm
var hwPrint=1;var hwDocHWID="nord420";var hwDocTitle="Alpha-Mannosidosis";var hwRank="1";var hwSectionHWID="nord420-Header";var hwSource="en-caQ2_05";var hwDocType="Nord";
National Organization for Rare Disorders, Inc.
Alpha-Mannosidosis
Important
It is possible that the main title of the report Alpha-Mannosidosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.
Synonyms
  • Lysosomal Alpha-D-Mannosidase Deficiency Alpha-Mannosidase B Deficiency Mannosidase, Alpha B, Lysosomal Mannosidosis Mannosidosis, Alpha B, Lysosomal
Disorder Subdivisions
  • None
General Discussion
Alpha-mannosidosis is one of a group of very rare inherited disorders known as glycoprotein and related storage diseases. These disorders are caused by a defect in the breakdown of complex molecules in the cells, as a result of an enzyme deficiency. The enzyme that is lacking is known as alpha-D-mannosidase. The molecules that are not broken down are stored in the small components within cells known as lysosomes. The approximately 50 diseases in which this occurs are known as lysosomal storage diseases. In addition to alpha mannosidosis and a related disorder, beta mannosidosis, the lysosomal storage disorders include Fabry disease, Gaucher disease, cystinosis, aspartylglycosaminuria, the mucopolysaccharidoses, the mucolipidoses, and others.
There are two types of alpha-mannosidosis: type I and type II. Type I is much less common. Its symptoms may begin within the first year of life. Type II, the milder form, may begin during the juvenile or adult years.

30. Alpha-Mannosidosis - St. Joseph Mercy, Ann Arbor Michigan
Alphamannosidosis - St. Joseph Mercy Health System Hospitals serving Ann Arbor, SE Michigan, Washtenaw County, Livingston County, Wayne County,
http://www.sjmercyhealth.org/14473.cfm

31. NORD - National Organization For Rare Disorders, Inc.
Alphamannosidosis is one of a group of very rare inherited disorders known as glycoprotein In addition to alpha mannosidosis and a related disorder,
http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Alpha-Mannosi

32. GeneReviews: Alpha-Mannosidosis
Your browser does not support HTML frames so you must view Alphamannosidosis in a slightly less readable form. Please follow this link to do so.
http://www.geneclinics.org/profiles/a-mannosidosis
Your browser does not support HTML frames so you must view Alpha-Mannosidosis in a slightly less readable form. Please follow this link to do so.

33. Mannosidosis
mannosidosis. Used for. mannosidase deficiency. Broader Terms. enzyme deficiency. Broader Terms. inborn carbohydrate metabolism disorder. Broader Terms
http://crisp.cit.nih.gov/Thesaurus/00004858.htm
Prev Term: mannosidase deficiency
Next Term: Mansonella ozzardi
mannosidosis
Used for:
mannosidase deficiency
Broader Terms:
enzyme deficiency
Broader Terms:
inborn carbohydrate metabolism disorder
Broader Terms:
inborn lysosomal enzyme disorder
Related Terms:
mannosidase
Related Terms:
mental retardation
Scope Note:
lysosomal storage disease due to defective alpha-mannosidase with resultant oligosaccharide accumulation.
Term Number:
Send your comments to: Melody Lowe

34. Mannosidosis
Complete online version of The Encyclopaedia of Medical Imaging including text and images from The Encyclopaedia of Medical Imaging s eight book volumes
http://www.amershamhealth.com/medcyclopaedia/medical/Volume VII/MANNOSIDOSIS.asp

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our commitment our company Search Medcyclopaedia for: Search marked text (mark text before you click) Browse entry words starting with: A B C D ... amershamhealth.com Mannosidosis, deficiency of alpha mannosidase which leads to accumulation of glycoproteins in the tissues. Clinically, children have craniofacial dysmorphism, psychomotor retardation, cataracts and hearing loss. Radiologically, the features mimic those of mucopolysaccharidosis. There is brachycephaly, sometimes craniostenosis, widened ribs, flattened vertebrae with anterior beaking at the thoracolumbar junction, widening of the tubular bones with some irregularity of the metaphyses, a narrow basal segment to the iliac wings and osteoporosis.
HC
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35. SAS Centre : Assays : Genetic Enzymes : B-Mannosidosis
b mannosidosis is a very rare disorder with only 10 cases in 7 families being known (Oct 1995). Clinical features vary widely but there has always been
http://www.sas-centre.org/assays/genetic_enzymes/lysstodisbetamannosidosis.html
Supra-Regional Assay Service
Centres for Analysis and Clinical Interpretation Assays home about us specialities centres ... Genetic Enzymes b -Mannosidosis
b -mannosidosis is a very rare disorder with only 10 cases in 7 families being known (Oct 1995). Clinical features vary widely but there has always been mental retardation. There may be some dysmorphism, dysotosis multiplex and angiokeratoma (the latter seen only in two brothers). One case only was particularly severe.
ENZYME TESTS: Deficiency of b -mannosidase is the primary defect.
Back to Alphabetical List of Assays Available
Top of Page Website designed by Paul Littlefield

36. SAS Centre : Assays : Genetic Enzymes : A-Mannosidosis
Assays / Genetic Enzymes / amannosidosis. Broadly, the disease can be sub-divided into mild and severe types, both very rare, although there is probably a
http://www.sas-centre.org/assays/genetic_enzymes/lysstodisamannosidosis.html
Supra-Regional Assay Service
Centres for Analysis and Clinical Interpretation Assays home about us specialities centres ... Genetic Enzymes a -Mannosidosis
Broadly, the disease can be sub-divided into mild and severe types, both very rare, although there is probably a continuum of phenotypes. In the most severe type there is hepatosplenomegaly, severe dysostosis multiplex, deafness, progressive mental retardation and often early death. In the milder form there is a later onset and more prolonged course with less severe dysostosis multiplex and mental retardation, but deafness is a major problem.
Enzyme Tests:
Deficiency of lysosomal a -mannosidase is the primary defect.
Back to Lysosomal Storage Disorders Index

Back to Alphabetical List of Assays Available
Top of Page Website designed by Paul Littlefield

37. Mannosidosis Articles, Support Groups, And Resources
mannosidosis articles, support groups, and resources for patients from Med Help International (www.medhelp.org)
http://www.medhelp.org/HealthTopics/Mannosidosis.html
[Health Topics A-Z]
A
B C D ... Z
Mannosidosis
Med Help International Search Medical Forums / Message Boards ... Site Map
Revised: 9/7/2005

38. Beta-Mannosidosis
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39. Mannosidosis In Galloway Calves -- Embury And Jerrett 22 (6): 548 -- Veterinary
mannosidosis in Galloway calves. DH Embury and IV Jerrett. mannosidosis was diagnosed in four stillborn Galloway calves and an autolyzed fullterm fetus
http://www.vetpathology.org/cgi/content/abstract/22/6/548

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ARTICLES
Mannosidosis in Galloway calves
D. H. Embury and I. V. Jerrett
Mannosidosis was diagnosed in four stillborn Galloway calves and an autolyzed full-term fetus from experimental matings of carrier animals. Gross lesions were moderate internal hydrocephalus, and pallor and enlargement of the liver and kidneys and arthrogryposis. Histologic changes in the central nervous system of each calf were marked foamy vacuolation of the cytoplasm of neurones in the cerebral cortex, thalamus and brainstem, and vacuolation of the Purkinje cells of the cerebellum. Spheroids were common throughout the brain and there was also consistent severe foamy cytoplasmic vacuolation of renal tubular epithelial cells and hepatocytes.

40. Swainsonine Toxicosis Mimics Lectin Histochemistry Of Mannosidosis -- Alroy Et A
Swainsonine toxicosis mimics lectin histochemistry of mannosidosis. J. Alroy, U. Orgad, AA Ucci and VE Gavris. Cells affected by locoweed (Astragalus
http://www.vetpathology.org/cgi/content/abstract/22/4/311

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ARTICLES
Swainsonine toxicosis mimics lectin histochemistry of mannosidosis
J. Alroy, U. Orgad, A. A. Ucci and V. E. Gavris
Cells affected by locoweed (Astragalus lentiginosus) and Swainsona galegifolia toxicosis or mannosidosis exhibit similarities in their catabolism of N-linked glycoproteins and accumulation of cytoplasmic vacuoles. We used nine different biotinylated lectins as histochemical markers for specific sugars and avidin-biotin-peroxidase complex as a visualant to study the cells affected with these conditions. Since locoweed and Swainsona spp block mannosidase activity, we expected a similar lectin staining pattern in cells under these conditions as that seen in mannosidosis. Concanavalia ensiformis agglutinin, wheat germ agglutinin and

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