PMID- 7757942
OWN - NLM
STAT- MEDLINE
DCOM- 19950628
LR  - 20181130
IS  - 1122-9497 (Print)
IS  - 1122-9497 (Linking)
VI  - 27
IP  - 2
DP  - 1995 Apr
TI  - The effect of lysosomal storage diseases on secretory cells: an ultrastructural 
      study of pancreas as an example.
PG  - 143-60
AB  - The lysosome is the disposal and recycling center of eukaryotic cells. The 
      pancreas is an exocrine and endocrine organ. It contains different cell types 
      secreting enzymes, hormones, ions and fluid. The purpose of this study was to 
      demonstrate that the manifestation of the disease in individual cells depends on 
      the expression of the defected gene within the function of the cells. We have 
      thus compared the ultrastructural features of normal pancreas to pancreas 
      affected by eleven different lysosomal storage diseases in human, cats, dogs and 
      mice. The diseases studied include GM1- and GM2- gangliosidosis, 
      galactosialidosis, alpha-mannosidosis, mucopolysaccharidosis-I (MPS-I), 
      mucopolysaccharidosis-VI (MPS-VI), mucopolysaccharidosis-VII (MPS-VII), 
      mucolipidosis-IV (ML-IV), Niemann-Pick C, neuronal ceroid-lipofuscinosis, and an 
      undefined glycolipid storage disease. We demonstrated that morphological changes 
      are seen in some pancreatic cell types but not in others, even though the genetic 
      defect occurs in all cell types. Furthermore, we identified and characterized 
      primary and secondary changes in affected cells. The primary changes include 
      presence of numerous and/or enlarged secondary lysosomes laden with partially 
      degraded substrates. The secondary changes were associated with an abnormal 
      formation and maturation of zymogen or secretory granules. The mechanisms 
      contributing to the secondary changes are thought to be associated with altered 
      synthesis of various constituents of these granules.
FAU - Hammel, I
AU  - Hammel I
AD  - Department of Pathology, Sackler School of Medicine, Tel Aviv University, Ramat 
      Aviv, Israel.
FAU - Alroy, J
AU  - Alroy J
LA  - eng
PT  - Journal Article
PL  - Italy
TA  - J Submicrosc Cytol Pathol
JT  - Journal of submicroscopic cytology and pathology
JID - 8804312
SB  - IM
MH  - Animals
MH  - Cat Diseases/pathology
MH  - Cats
MH  - Dog Diseases/pathology
MH  - Dogs
MH  - Gangliosidosis, GM1/pathology
MH  - Humans
MH  - Lysosomal Storage Diseases/*pathology/veterinary
MH  - Lysosomes/*ultrastructure
MH  - Male
MH  - Mice
MH  - Microscopy, Electron
MH  - Mucolipidoses/pathology
MH  - Mucopolysaccharidoses/pathology
MH  - Pancreas/metabolism/*pathology/*ultrastructure
EDAT- 1995/04/01 00:00
MHDA- 1995/04/01 00:01
CRDT- 1995/04/01 00:00
PHST- 1995/04/01 00:00 [pubmed]
PHST- 1995/04/01 00:01 [medline]
PHST- 1995/04/01 00:00 [entrez]
PST - ppublish
SO  - J Submicrosc Cytol Pathol. 1995 Apr;27(2):143-60.