Similarity of Lectin Histochemistry of a Lysosomal Storage Disease in a New Zealand Lamb to that of Ovine GM1 Gangliosidosis

Autor: W. J. Hartley, D. J. Prieur, R. D. Murnane
Rok vydání: 1991
Předmět:
Zdroj: Veterinary Pathology. 28:332-335
ISSN: 1544-2217
0300-9858
Popis: Lysosom al storage diseases are caused by inherited defigangliosidos is has recentl y been characterized." A comparciencies of vario us lysosomal enzymes and result in the proati ve study oflectin bind ing pattern s was per formed on cengress ive sto rage of uncatab olizabl e substrate .v'v Th e specific tral nervou s system tissue from two lambs affected with ovi ne enzyme deficiency(ies) and residual activ ity of the deficient GM , gangliosidos is and a lam b affected with the disease seen enzy me(s) for natural subs trates determ ine which orga n is in New Zealand. (organs are) pr im aril y affected and, thu s, the clini cal presenLect in hi st och emi st ry was co nd uc ted as pr evi ou sly tati on of eac h lysosom al storage disease. T hey are usually described! on formalin-fixed and paraffin-embedd ed sectio ns inh erited as autosom al recessive disord ers and often result of cerebru m, cerebellum, and brain stem or spinal cord on in death du ring childhood. Th ere are ove r 30 human Iysothe following: fro m an 8-mo nth-o ld lamb (tissues in a 28soma l sto rage diseases, and an imal models of these di seases year-o ld paraffin block) affected with the disease seen in New represent a valuable reso urce for the development of new Zea land (New Zealand lamb); two 6-mo nth-old lambs afdiagnostics and therap euti cs and for furt her elucida tio n of fected with ov ine G M , gangliosidosis; and a 6-month-old lysosomal functio n. normal (contro l) lamb. Th e biotinylated lect ins and concenWe have recently descr ibed an autosoma l recessive lysotrat ion s used , maj or carbohydra te specificities of each lectin , soma l storage dise ase in Suffolk sheep with a pro found deand the binding inh ibit ors used are listed in Table I . Negati ve ficiency of lysosomal {1-galactos idase and partial deficiency contro ls consisted of sections incub ated with phosphate buffofa -neuram inidase that result s in the death of affected lambs ered saline alone instead oflectins. Incub at ion of eac h lectin at 4 to 6 mon th s of age.v" Th ough there is a dual enzyme with its correspo nd ing binding inhibito r before applicatio n deficiency, thi s ovi ne disease is homo logous with human to a match ing set of slides served as con tro ls for binding G M, gangliosidosis (deficie ncy of{1-galactosidase) and is not specificity . hom ologou s with human galactosia lidos is (de ficiencies of T he lectin binding pattern ofcen tra l nervous system tissue {1-galactosidase and a -neuraminidase ) based upon interspefrom the two lambs affected with ovi ne GM, gangliosido sis cific genetic complementation analysis of somatic cell hywas the same as previou sly describ ed' and was present in all brids. ' The disease has thus been term ed ov ine G M) ganareas of the central nervou s system. Th e cytoplasm of neugliosidosis. A simi lar di sease was seen in a gro up of relat ed rons (including Purkinje, Go lgi, and stellate basket cells and 8-month-o ld lambs on one farm in New Zealand ov er 28 cells of the mo lecular layer of the cere bellum) stai ned with years ago an d termed, " lipid-like neuronal degenerati on. " ? peanut (Arachis hypogea) agglutinin (PNA ), castor bean (RicAs with ovi ne G M , ganglios idosis, lambs suddenly develinus com m unis) agglutinin-I (RCA-I), horse gram (Dolichos oped atax ia that rapidly progressed to prostration . Lesion s bijlorus) agglutinin (DBA), soybea n (Glycine max ) agglut inin in the centra l nervous system were character istic of a neu(SBA), and horseshoe crab (Limulus polyphemus) agglutinin ronal lysosom al storage disease: there was diffuse enlargeto a substantia lly great er degree than the neuronal cytoplasm ment and vac uolatio n of virtually all neuron s; and the lesions of contro l sheep. Th e central nervou s system tissue of the were identical to those seen in ovi ne G M , gangliosidosis New Zealand lamb had a nearl y identica l lectin bind ing pat(Figs. I , 2). tern as that observe d in the two lamb s affected with ovi ne Lectin histochemi stry has been used for the charact erizaG M , gangliosidosis (Figs. 3-8). Th e onl y differences were tion of sto red products in many lysosom al sto rage diseases. stro ng binding of LPA by neuron s from the New Zealand Lectin s are agglut inating proteins with well-characterized and lamb versus slight binding by neuron s from the two lambs specific affi nities for carbo hydrates and, when bound to visaffected with ovi ne G M , gangliosidos is, and slight to negative ualant s, allow localizati on and ident ificat ion of carbohybind ing of WGA by neurons fro m the New Zealand lamb, drat es in tissues. Unique lecti n bindi ng pattern s have been vers us stro ng to slight binding of WGA by neuro ns from the observed for different lysosom al sto rage diseases, and simi lar two lambs affected with ovi ne G M ) gangliosidosis. Incubapatterns have been observed for the same sto rage disease in tion of each lectin with its corresponding inhibitor consisd ifferent species. Th e lectin bind ing pattern of ovi ne G M , tentl y decreased or blocked bind ing of the lectin to tissue
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