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

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
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