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Beta-mannosidosis

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Sandra Baxendell
Sandra Baxendell

Beta-mannosidosis is a genetic disease of Anglo-Nubians and Nubians. There is no cure. Kids are born unable to stand and with nodding heads and front legs unable to straighten

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AGDEX 470 654-' Fl9l/sEP 83 DAIRY GOATS - beta-mannosidosis of Anslo-Nubians by S.A. BaxendeIl, 0airy CattIe Husbandry Branch, l{erstead A lysosomol storoge diseose of Anglo-Nubion kids wos first recorded in New South Woles in 1973. A storoge diseose usuolly occurs when on onimol locks o potticulol enzyme so thot o biochemicol leoction in the onimol's body connot toke PJ.occ. This meons thot there is o mossive build up of substonces thot hove to be stored bccouse they connot be converted due to the deficiency of the enzyme. These substonces oie stored in Iysosornes, or membrone Iincd spoces within the onlmol's cells. In 198 l, Anericon reseorchers found thot offectcd nubion kids lockcd thc cnzymc beto-monnos idose, ond loter thot yeor Austrolion reseo iche is confirmed this condition in Anglo- Nubion kids in Ncw South Woles. CLINICAL SIGNS Affected kids one born olive but ore unoble to stond becouse they lock the obility to stroighten their Iegs. These kids ore bright ond olert ond hove o good sucking reflex. Although oble to move their legs, heod ond toiI, the kids suffer from neck trdnors resulting in o wobbling movement of the heod. Affected kids hove to be destroyed. THE POST-MORTEM EXAT{INATION Nothing is evident qt post{ortem, however, when specimens ore exomined pnder o microscope ot o loter dote, o typicoL storoge diseose pictune emerges. Cells in the bnoin, kidney ond othen orcos or.e so full of Iysosomes contoining the products thot should hovc bcen convertcd by the missing enzyme thot there is vcry l ittle celluIor moteriol left. Biochemicol studtes show o deficiency of the enzyme beto<nonnosido se in the tissues ond the blood of offected kids. HOIT IS THIS DISEASE TRAilSMIITED? Beto-monnos idos i s Iike rnos t othcr lysosonol stofog€ diseos€b is inherited ln on outosofllo I icccssive monnci. Affected kids ore only born when o.cortier buck is motcd to o corrier'doe, os shown in figurc l. A corlier hos holf the normol levcls of beto-monnos idose in its blood ond con be identified by o blood test. HOIV CAN BETA-MANNOS I DOS I S BE ffi The eosicst woy to control ony inheri ted lysosanol storoge diseose. including beto<nonnos idos i s is to moke certoin thot oIl Anglo-Nubion bucks used os sires ore blood tested ond orc not coirieis of bcto- monnosidosis. This one step will ensurs thot no offected kids wlll bc boln . Pure bred herds thot produc-. stock for sole should hove thelr stock tested to ensuie thot ony goot sold beoring their stud prefix ore not cot.riGl.s. Prospectlve purchoscrs of Anglo-Nubions. especiol ly bucks, Cucensland Dcpartrent of Prirary Industrics, G.P.0. Bor 46, Erisbanc' 0. 4001.
would b€ weII odvised to rque3t o vet€rinory csatificote to confi nn thot the stock for sole hos bcen blood tested ond is normol or thot thby ore the offspring of tested norrno I po rents . Figure L -t-- Vlhen o cor'Fier buck is used ovGr' normol does, 50% of th6 kids orc normol ond 50% ore coiriers os shown in Figure 2. Prolonged use of normol Anglo-Nubion bucks will rcducc the incidence of corrieFs in tha overoll populotion to vet.y low lcvcls. doe moy produce I normol, 2' doe nro y produce 2 normol, kids ond ) A co rriar buck corrigr ond I moted to o corricr offccted kld. F igurc 2. corrier buck moted to o normol co rrier klds. A 2 DC83/6

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Beta-mannosidosis

  • 1. AGDEX 470 654-' Fl9l/sEP 83 DAIRY GOATS - beta-mannosidosis of Anslo-Nubians by S.A. BaxendeIl, 0airy CattIe Husbandry Branch, l{erstead A lysosomol storoge diseose of Anglo-Nubion kids wos first recorded in New South Woles in 1973. A storoge diseose usuolly occurs when on onimol locks o potticulol enzyme so thot o biochemicol leoction in the onimol's body connot toke PJ.occ. This meons thot there is o mossive build up of substonces thot hove to be stored bccouse they connot be converted due to the deficiency of the enzyme. These substonces oie stored in Iysosornes, or membrone Iincd spoces within the onlmol's cells. In 198 l, Anericon reseorchers found thot offectcd nubion kids lockcd thc cnzymc beto-monnos idose, ond loter thot yeor Austrolion reseo iche is confirmed this condition in Anglo- Nubion kids in Ncw South Woles. CLINICAL SIGNS Affected kids one born olive but ore unoble to stond becouse they lock the obility to stroighten their Iegs. These kids ore bright ond olert ond hove o good sucking reflex. Although oble to move their legs, heod ond toiI, the kids suffer from neck trdnors resulting in o wobbling movement of the heod. Affected kids hove to be destroyed. THE POST-MORTEM EXAT{INATION Nothing is evident qt post{ortem, however, when specimens ore exomined pnder o microscope ot o loter dote, o typicoL storoge diseose pictune emerges. Cells in the bnoin, kidney ond othen orcos or.e so full of Iysosomes contoining the products thot should hovc bcen convertcd by the missing enzyme thot there is vcry l ittle celluIor moteriol left. Biochemicol studtes show o deficiency of the enzyme beto<nonnosido se in the tissues ond the blood of offected kids. HOIT IS THIS DISEASE TRAilSMIITED? Beto-monnos idos i s Iike rnos t othcr lysosonol stofog€ diseos€b is inherited ln on outosofllo I icccssive monnci. Affected kids ore only born when o.cortier buck is motcd to o corrier'doe, os shown in figurc l. A corlier hos holf the normol levcls of beto-monnos idose in its blood ond con be identified by o blood test. HOIV CAN BETA-MANNOS I DOS I S BE ffi The eosicst woy to control ony inheri ted lysosanol storoge diseose. including beto<nonnos idos i s is to moke certoin thot oIl Anglo-Nubion bucks used os sires ore blood tested ond orc not coirieis of bcto- monnosidosis. This one step will ensurs thot no offected kids wlll bc boln . Pure bred herds thot produc-. stock for sole should hove thelr stock tested to ensuie thot ony goot sold beoring their stud prefix ore not cot.riGl.s. Prospectlve purchoscrs of Anglo-Nubions. especiol ly bucks, Cucensland Dcpartrent of Prirary Industrics, G.P.0. Bor 46, Erisbanc' 0. 4001.
  • 2. would b€ weII odvised to rque3t o vet€rinory csatificote to confi nn thot the stock for sole hos bcen blood tested ond is normol or thot thby ore the offspring of tested norrno I po rents . Figure L -t-- Vlhen o cor'Fier buck is used ovGr' normol does, 50% of th6 kids orc normol ond 50% ore coiriers os shown in Figure 2. Prolonged use of normol Anglo-Nubion bucks will rcducc the incidence of corrieFs in tha overoll populotion to vet.y low lcvcls. doe moy produce I normol, 2' doe nro y produce 2 normol, kids ond ) A co rriar buck corrigr ond I moted to o corricr offccted kld. F igurc 2. corrier buck moted to o normol co rrier klds. A 2 DC83/6