Difference between revisions of "Lysosomal Storage Disease"

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==Description==
 
==Description==
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Lysosomal storage diseases occur due to inherited deficiencies of various lysosomal enzymes, either due to abnormalities in its synthesis, processing or cellular trafficking.  The normal function of lysosomes is to degrade molecules within the cell and, when these chemical reactions are truncated by the absence of a key enzyme, the substrates of the reaction accumulate within the organelle.  Since there is some redundancy in the pathways by which proteins are degraded, deficiencies in proteases do not usually result in clinical disease.
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Lysosomal storage diseases occur due to inherited deficiencies of various lysosomal enzymes and this may be due to abnormalities in their synthesis, processing or cellular trafficking.  The normal function of lysosomes is to degrade molecules within the cell and, when these chemical reactions are truncated by the absence of a key enzyme, the substrates of the reaction accumulate within the organelle.  Since there is some redundancy in the pathways by which proteins are degraded, deficiencies in proteases do not usually result in clinical disease.  Deficiencies in the nuclease enzymes are also not usually compatible with survival.
  
 
There are a large number of different lysosomal storage diseases in dogs and cats categorised according to the product which accumulates within cells.  All of the storage disease are rare.   
 
There are a large number of different lysosomal storage diseases in dogs and cats categorised according to the product which accumulates within cells.  All of the storage disease are rare.   
  
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===Classification===
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{|cellpadding="10" cellspacing="0" border="1"
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| '''Class'''
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| '''Defective Enzyme'''
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| '''Name of Disease'''
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|-
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| '''Sphingolipidosis'''
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| beta D gangliosidase<br>
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beta D hexosaminidase<br>
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beta D glucocerebrosidase<br>
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beta D galactocerebrosidase<br>
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Sphingomyelinase
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| GM1 Gangliosidosis<br>
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GM2 Gangliosidosis<br>
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Gaucher Disease<br>
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Krabbe Disease<br>
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Niemann-Pick Disease
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|-
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| '''Oligosaccharidosis'''
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|
 +
|
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|-
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| '''Mucopolysaccharidosis'''
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|
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|
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|-
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| '''Glycoproteinosis'''
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|
 +
|
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|-
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| '''Proteosis'''
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|
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|
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|}
  
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===Pathophysiology===
  
 
[[Category:Liver - Storage Diseases]]
 
[[Category:Liver - Storage Diseases]]
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[[Category:To_Do_-_James]]
 
[[Category:To_Do_-_James]]
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[[Category:Dog]][[Category:Cat]]

Revision as of 10:38, 28 July 2010


 This article is still under construction.


Description

Lysosomal storage diseases occur due to inherited deficiencies of various lysosomal enzymes and this may be due to abnormalities in their synthesis, processing or cellular trafficking. The normal function of lysosomes is to degrade molecules within the cell and, when these chemical reactions are truncated by the absence of a key enzyme, the substrates of the reaction accumulate within the organelle. Since there is some redundancy in the pathways by which proteins are degraded, deficiencies in proteases do not usually result in clinical disease. Deficiencies in the nuclease enzymes are also not usually compatible with survival.

There are a large number of different lysosomal storage diseases in dogs and cats categorised according to the product which accumulates within cells. All of the storage disease are rare.

Classification

Class Defective Enzyme Name of Disease
Sphingolipidosis beta D gangliosidase

beta D hexosaminidase
beta D glucocerebrosidase
beta D galactocerebrosidase
Sphingomyelinase

GM1 Gangliosidosis

GM2 Gangliosidosis
Gaucher Disease
Krabbe Disease
Niemann-Pick Disease

Oligosaccharidosis
Mucopolysaccharidosis
Glycoproteinosis
Proteosis

Pathophysiology

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