Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis

J. Alroy; A. Bachrach; J. G. Thalhammer; N. Panjwani; R. Richard; R. DeGasperi; C. D. Warren; D. M. Albert; S. S. Raghavan

doi:10.1007/BF02899544

Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis

J. Alroy, A. Bachrach, J. G. Thalhammer, N. Panjwani, R. Richard, R. DeGasperi, C. D. Warren, D. M. Albert, S. S. Raghavan

Research output: Contribution to journal › Article › peer-review

6 Scopus citations

Abstract

The clinical, neurophysiological, morphological and biochemical manifestations of eyes from Persian kittens affected with α-mannosidosis were studied. Clinically the disease is characterized by progressive corneal and lenticular opacification. In addition there is asymmetry in shape and latency of signal conductions which were demonstrated by visual evoked potential studies. Morphological and histochemical studies revealed vacuolization of various ocular cell types which stained positively with Concanavalia ensiformis agglutinin (Con A) and wheat germ agglutinin (WGA). Biochemical studies illustrated low activity of acid α-mannosidase in cultured keratocytes and abnormal storage of partially degraded oligosaccharides in these cells, in vitreous humor and lens. This comprehensive study of ocular α- mannosidosis demonstrates enzyme deficiency which leads to abnormal storage of oligosaccharides in affected cells and is manifested by morphological alterations and functional impairment.

Original language	English (US)
Pages (from-to)	173-180
Number of pages	8
Journal	Virchows Archiv B Cell Pathology Including Molecular Pathology
Volume	60
Issue number	1
DOIs	https://doi.org/10.1007/BF02899544
State	Published - Dec 1991
Externally published	Yes

Keywords

Cats eyes
α-Mannosidosis

ASJC Scopus subject areas

Pathology and Forensic Medicine

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10.1007/BF02899544

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title = "Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis",

abstract = "The clinical, neurophysiological, morphological and biochemical manifestations of eyes from Persian kittens affected with α-mannosidosis were studied. Clinically the disease is characterized by progressive corneal and lenticular opacification. In addition there is asymmetry in shape and latency of signal conductions which were demonstrated by visual evoked potential studies. Morphological and histochemical studies revealed vacuolization of various ocular cell types which stained positively with Concanavalia ensiformis agglutinin (Con A) and wheat germ agglutinin (WGA). Biochemical studies illustrated low activity of acid α-mannosidase in cultured keratocytes and abnormal storage of partially degraded oligosaccharides in these cells, in vitreous humor and lens. This comprehensive study of ocular α- mannosidosis demonstrates enzyme deficiency which leads to abnormal storage of oligosaccharides in affected cells and is manifested by morphological alterations and functional impairment.",

keywords = "Cats eyes, α-Mannosidosis",

author = "J. Alroy and A. Bachrach and Thalhammer, {J. G.} and N. Panjwani and R. Richard and R. DeGasperi and Warren, {C. D.} and Albert, {D. M.} and Raghavan, {S. S.}",

year = "1991",

month = dec,

doi = "10.1007/BF02899544",

language = "English (US)",

volume = "60",

pages = "173--180",

journal = "Virchows Archiv B Cell Pathology Including Molecular Pathology",

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T1 - Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis

AU - Alroy, J.

AU - Bachrach, A.

AU - Thalhammer, J. G.

AU - Panjwani, N.

AU - Richard, R.

AU - DeGasperi, R.

AU - Warren, C. D.

AU - Albert, D. M.

AU - Raghavan, S. S.

PY - 1991/12

Y1 - 1991/12

N2 - The clinical, neurophysiological, morphological and biochemical manifestations of eyes from Persian kittens affected with α-mannosidosis were studied. Clinically the disease is characterized by progressive corneal and lenticular opacification. In addition there is asymmetry in shape and latency of signal conductions which were demonstrated by visual evoked potential studies. Morphological and histochemical studies revealed vacuolization of various ocular cell types which stained positively with Concanavalia ensiformis agglutinin (Con A) and wheat germ agglutinin (WGA). Biochemical studies illustrated low activity of acid α-mannosidase in cultured keratocytes and abnormal storage of partially degraded oligosaccharides in these cells, in vitreous humor and lens. This comprehensive study of ocular α- mannosidosis demonstrates enzyme deficiency which leads to abnormal storage of oligosaccharides in affected cells and is manifested by morphological alterations and functional impairment.

AB - The clinical, neurophysiological, morphological and biochemical manifestations of eyes from Persian kittens affected with α-mannosidosis were studied. Clinically the disease is characterized by progressive corneal and lenticular opacification. In addition there is asymmetry in shape and latency of signal conductions which were demonstrated by visual evoked potential studies. Morphological and histochemical studies revealed vacuolization of various ocular cell types which stained positively with Concanavalia ensiformis agglutinin (Con A) and wheat germ agglutinin (WGA). Biochemical studies illustrated low activity of acid α-mannosidase in cultured keratocytes and abnormal storage of partially degraded oligosaccharides in these cells, in vitreous humor and lens. This comprehensive study of ocular α- mannosidosis demonstrates enzyme deficiency which leads to abnormal storage of oligosaccharides in affected cells and is manifested by morphological alterations and functional impairment.

KW - Cats eyes

KW - α-Mannosidosis

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JO - Virchows Archiv B Cell Pathology Including Molecular Pathology

JF - Virchows Archiv B Cell Pathology Including Molecular Pathology

IS - 1

ER -

Clinical, neurophysiological, biochemical and morphological features of eyes in Persian cats with mannosidosis

Abstract

Keywords

ASJC Scopus subject areas

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