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John C. Fyfe, D.V.M, Ph.D.
Associate Professor D.V.M., 1984, Washington-Oregon-Idaho Regional Program in Veterinary Medicine Ph.D., 1994, University of Pennsylvania Department of Microbiology and Molecular Genetics 5169 Biomedical Physical Sciences Michigan State University East Lansing, MI 48824 Email: fyfe@cvm.msu.edu
Glycogen storage disease type IIIa (GSDIIIa) is an inherited metabolic disorder that causes liver and skeletal muscle disease due to deficiency of the glycogen
debranching enzyme (GDE) and tissue storage of abnormally structured glycogen. This disorder was discovered in an extended family of curly coated retrievers
(CCR), with representatives from USA, Canada, and New Zealand, and is due to a single based deletion in the GDE gene. This disease, too, is an autosomal
recessive trait, and so the laboratory provides carrier testing for GSD IIIa in curly coated
retrievers .
A quick explanation is shown in the tables below. BB being a dog that does not have the disease and does not carry the recessive gene for GSDIIIa. Bb demonstrates a dog that does not have the disease, but does carry the recessive gene. bb represents a dog that is affected with the disease. Dogs that are carriers need not be eliminated from breeding. Carriers bred to clear, non carriers, will produce some carriers and some non-carriers. These offspring may then be tested and used as valuable animals in a breeding program.
Think in terms of a stop light. Green means go... a non carrier dog, you can breed. Orange means caution... a dog that is a carrier, can be bred, but only to
non-carriers. Red means STOP! this is a dog with the disease. Even if it could be bred, all pups will be carriers.
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produces all clear, not carrier offspring(BB)
The offspring af a breeding like this would all be clear of GSD based on the absence of the GSD mutation in both parents. |
A non affected, not carrier dog (BB) A non affected carrier bitch (Bb).
produces all non affected pups, two of which would carry the GSDIIIa gene as a recessive trait (Bb) No dogs would have the disease, but all offspring should be tested prior to breeding |
one GSDIIIa affected pup(bb) and 2 pups that are carriers(Bb) There is the potential for pups that will have Glycogen storage disease, and some will be carriers.
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All pups are carriers, and should be only bred to non-carriers. |
Some of the pups will have Glycogen storage disease, and some will be carriers. |
These are just examples. I do not think anyone would breed two affected dogs.
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SoftMaple GSDIIIa results:
DOB 4/10/2002 out of CH SoftMaple's International Fling CGC CCR 91 X CH SoftMaple's O' Dark Thirty JH SH CD WC WCX CGC HOF CCR 92
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DOB 11/8/2004 CH SoftMaple's O'Dark Thirty JH, SH, MH, WC, WCX, WCQ, CD, CGC TT, HOF CCR 92 And SoftMaple N HunterBay's Poetic Justice CGC Carrier CCR 105
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DOB: 12/10/2002 SJCH, SUCH Ringlets Constant Wind X CH SoftMaple's Fairway Explorer CGC CCR 150 The offspring from this litter would not be assumed clear. One of the parents must be a carrier to have produced carriers.
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DOB 5/9/2005 Ch. Dese's Black As Coal, C D, WC, CGC, TT, JH, RN CCR 90) CH SoftMaple's Pheasant Dreams WC, CGC, RN CCR 106
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DOB: 9/29/1997 Ch. Avanti's Best Bet CD ,CGC, ROMX GSDIIIa clear X CH Riverwatch DesertWind ROM CCR 103
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DOB 11/8/2004 CH SoftMaple's O'Dark Thirty JH, SH, MH, WC, WCX, WCQ, CD, CGC TT, HOF CCR 92 And CH Charwin Evensong WCX, JH, CGC, CD CCR 115
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5/16/2006 SHR, CH Elflock False Echoes WC CCR 146 & CH SoftMaple's Fairway Explorer CGC CCR 150
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DOB 09/08/04 CH SoftMaple N HunterBay's NYwkend CGC TT CCR 143 And CH SR Curlygleann Kiwi Dream WC CGC TDI CCR#CCR202
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CH Soft Maple Water Works CGC CCR 107 CH SHR SoftMaple's Ma Belle Bouclee WC, WCX, JH, SH, CGC CCR 79 SoftMaple Cimramin Journeyman CCR 53 U-AG1 Softmaple Frolic In The Wind NJC, TN-N, TG-N, TN-O, RN CCR 51 CH SoftMaple's O' Dark Thirty JH, SH, MH, CD, WC, WCX, WCQ, CGC, TT, HOF CCR 92 CH SoftMaple's Pheasant Dreams WC, CGC, RN CCR 106 CH Soft Maple Boyerie Int'l Dateline CCR 132 CH Softmaple'Nhunterbay's NYwkend CGC CCR 143 Ch Fairway's Softmaple Finnheir, CGC, CD,NA, NAJ, RN, RA (clear, need to find out his number!) Soft Maple Singin The Blues CCR-118 CH SoftMaple's Fairway Explorer CGC CCR 150 Hntrbay's O'Dark Justice CCR 145 SoftMaple N HunterBay's Poetic Justice CGC Carrier CCR 105 CH SoftMaple's Comin' To America CGC CD -(Carrier) CCR 149
*GSDIIIa Pending means that the swabs have been sent into Michigan State University and we are awaiting results.
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DOB: 1/3/2005 Co-bred with Gunflint Curlies out of Boyerie Dese Maestro Music Please CHIC CCR 75 and CH Soft Maple's Gunflint Beauty CGC, CHIC (GSDIIIa clear by cleared parents)
SR23711801 Softmaple Yankee Pudle
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DOB: Sep 25 2002 U-CH, UAG1, CH Ptarmigan Huron's Soft Echo CGC (Carrier) X CH Aberbran Bar Von Bern JH, SH, WC, WCX, ROM (GSDIIIa status not currently on file) The offspring from this litter would not be assumed clear. The resulting litter produced both carriers and affected animals.
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Special thanks go to Molly Kirwan, without whom the GSDIIIa DNA test would not be in place.
DOB: Apr 7 1999 Ch. Avanti's Best Bet CD ,CGC, ROMX GSDIIIa clear X Ch. Ptarmigan Sun Devil, JH, WC, WCX, CD(Deceased age 12 before GSDIIIa test) The offspring from this litter would not be assumed clear, since they had one parent that must have been a carrier.
U-CH, UAG1, CH Ptarmigan Huron's Soft Echo CGC (Carrier)
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