Fainting Goats
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The fainting goat or myotonic goat is an American
breed A breed is a specific group of breedable domestic animals having homogeneous appearance (phenotype), homogeneous behavior, and/or other characteristics that distinguish it from other organisms of the same species. In literature, there exist seve ...
of
goat The goat or domestic goat (''Capra hircus'') is a species of Caprinae, goat-antelope that is mostly kept as livestock. It was domesticated from the wild goat (''C. aegagrus'') of Southwest Asia and Eastern Europe. The goat is a member of the ...
. It is characterised by
myotonia congenita ''Myotonia congenita'' is a congenital neuromuscular channelopathy that affects skeletal muscles (muscles used for movement). It is a genetic disorder. The hallmark of the disease is the failure of initiated contraction to terminate, often refe ...
, a hereditary condition that may cause it to stiffen or fall over when excited or startled.Martin, A. F., Bryant, S. H., & Mandel, F. (1984). Isomyosin distribution in skeletal muscles of normal and myotonic goats. Muscle & Nerve, 7(2), 152–160. It may also be known as the Tennessee fainting goat, falling goat, stiff-legged goat or nervous goat, or as the Tennessee wooden-leg goat. Four goats of this type were brought to
Tennessee Tennessee (, ), officially the State of Tennessee, is a landlocked U.S. state, state in the Southeastern United States, Southeastern region of the United States. It borders Kentucky to the north, Virginia to the northeast, North Carolina t ...
in the 1880s.


History

Fainting goats were first brought to
Marshall County, Tennessee Marshall County is a county A county () is a geographic region of a country used for administrative or other purposesL. Brookes (ed.) '' Chambers Dictionary''. Edinburgh: Chambers Harrap Publishers Ltd, 2005. in some nations. The term is der ...
, in the 1880s. Existing breeds of myotonic goats seem to have originated from a limited number of goats in Tennessee in the 1880s. White and Plaskett reported seeing these goats in five counties in Tennessee: Marshall, Giles, Lawrence, Maury, and Coffee. There were also goats in Texas that were brought over from Giles County in Tennessee, solely for a farmer who claimed to know of the goats' existence to prove it as fact to his neighbors. The goats were unable to jump over normal-sized fences, and found holes in the ground to crawl underneath the fences, similar to
pig The pig (''Sus domesticus''), also called swine (: swine) or hog, is an omnivorous, domesticated, even-toed, hoofed mammal. It is named the domestic pig when distinguishing it from other members of the genus '' Sus''. Some authorities cons ...
s. This unusual behavior made the goats more desirable in this era, as many farmers used stone walls for fences, therefore containing their goats. In Marshall county, there was also a buck goat brought over from Canada. The A. & M. College in Texas owned a zoo during 1926-1927 in which a myotonic goat was presented. Dr. White (in a letter to the author) stated that in the summer of 1929 in Egypt, he witnessed several fainting goats between the Suez Canal and the Palestine border. He also stated that he shipped some of the goats from Tennessee to a professor by the name of Nagel, at the Nervous Disease Institute in Germany for studies. The fainting was first described in scientific literature in 1904 and described as a "congenital myotonia" in 1939.Rüdel, R (2000). "Muscle chloride channelopathies: myotonia congenita". In Lehmann-Horn, Frank; Jurkat-Rott, Karin (eds.). ''Channelopathies''. Burlington: Elsevier. pp. 44–46. . The mutation in the goat gene that causes this muscle stiffness was discovered in 1996, several years after the equivalent gene had been discovered in humans and mice. The tendency of goats to spasm has been attested as early as the
Hippocratic Corpus The Hippocratic Corpus (Latin: ''Corpus Hippocraticum''), or Hippocratic Collection, is a collection of around 60 early Ancient Greek medical works strongly associated with the physician Hippocrates and his teachings. The Hippocratic Corpus cov ...
, where analogies are drawn from the phenomenon to human illness. The experiments of Brown and Harvey in 1939 with the myotonic goat made a major contribution to the understanding of the physiological basis of this condition and influenced many other theories of myotonia and its causes. The myotonic goat is important in history for researching and clarifying the role of chloride in muscle excitation.


Characteristics

Myotonic goats vary heavily in their characteristics in comparison to other goat breeds, and they possess certain traits that other goat breeds do not. Distinctly, their head and body formation tends to be different. Myotonic goats have a wide body and a heavier mass. The muscle condition of the myotonic goats usually leads to an increased muscle mass with a broader build. Slightly smaller than standard breeds of the goat, fainting goats are generally to tall and can weigh anywhere from to . Males, billies, or bucks as they are often referred to, can be as heavy as . Broadness is shown throughout the back and shoulders, due to muscle density. Myotonic goats have large, prominent eyes in high sockets that tend to protrude and are fairly distinct features. They are sometimes called "bug-eyed" for this feature. The heads tend to be medium length with a broad muzzle. Jaws tend to be broad as well, and distinct. The face is usually straight or convex. The ears tend to be normal-sized and closer to the face. The ears also exhibit a ripple halfway down the length of the ear. The horns tend to run large and have between. The neck tends to be muscle dense and more round than that of dairy breeds. The skin on many male's necks is wrinkled and thick. The neck can also run horizontally and, therefore, the head can be lower. Their hair can be short or long, with certain individuals producing a great deal of cashmere during colder months. Coats can demonstrate any color or pattern. Myotonic goats are vigilant animals that are quieter than other goat breeds. Other important differential characteristics of the myotonic goat include their high trainability, stiffness, high quality adaptation to low-input farm land and foraging, and cross-breeding creating hybrids leading to physical strength and good health.


Cause of "fainting"

Myotonia congenita is caused by an inherited disorder of a
chloride channel Chloride channels are a superfamily of poorly understood ion channels specific for chloride. These channels may conduct many different ions, but are named for chloride because its concentration ''in vivo'' is much higher than other anions. Several ...
in the
muscles Muscle is a soft tissue, one of the four basic types of animal tissue. There are three types of muscle tissue in vertebrates: skeletal muscle, cardiac muscle, and smooth muscle. Muscle tissue gives skeletal muscles the ability to muscle contra ...
of the skeleton (skeletal muscle chloride channel 1,
CLCN1 The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regul ...
).Constable, PD; Hinchcliff, KW; Done, SH; Gruenberg, W. "Inherited diseases of muscle". ''Veterinary Medicine – A textbook of the diseases of cattle, horses, sheep, pigs and goats'' (11th ed.). Elsevier. pp. 1514–30. . Congenital myotonia can be inherited as an
autosomal dominant In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the Phenotype, effect of a different variant of the same gene on Homologous chromosome, the other copy of the chromosome. The firs ...
trait (with
incomplete penetrance Penetrance in genetics is the proportion of individuals carrying a particular variant (or allele) of a gene (genotype) that also expresses an associated trait (phenotype). In medical genetics, the penetrance of a disease-causing mutation is the pr ...
) or a
recessive trait In genetics, dominance is the phenomenon of one variant (allele) of a gene on a chromosome masking or overriding the effect of a different variant of the same gene on the other copy of the chromosome. The first variant is termed dominant and ...
, resulting in the varying severity of the condition.Beck, C. L., Fahlke, C., & George, A. L. (1996). Molecular basis for decreased muscle chloride conductance in the myotonic goat. Proceedings of the National Academy of Sciences, 93(20), 11248-11252. Smith, BP, ed. (2015). "Chapter 42: Diseases of muscle. Myotonia". ''Large animal internal medicine''(5th ed.). Mosby. p. 1281. . In affected goats, the CLCN1 gene contains a
missense mutation In genetics, a missense mutation is a point mutation in which a single nucleotide change results in a codon that codes for a different amino acid. It is a type of nonsynonymous substitution. Missense mutations change amino acids, which in turn alt ...
; the amino acid
alanine Alanine (symbol Ala or A), or α-alanine, is an α-amino acid that is used in the biosynthesis of proteins. It contains an amine group and a carboxylic acid group, both attached to the central carbon atom which also carries a methyl group sid ...
is replaced with a
proline Proline (symbol Pro or P) is an organic acid classed as a proteinogenic amino acid (used in the biosynthesis of proteins), although it does not contain the amino group but is rather a secondary amine. The secondary amine nitrogen is in the p ...
residue. This small change causes the chloride channel in the muscle fibres to have a reduced conductance of chloride ions. This missense mutation occurs in a sequence of seven amino acids that are included in a group of closely related channels including that of humans and rats. From the study above, it was found that a change in the nucleotide sequence caused a proline substitution for alanine residue in the carboxyl terminus of the goat's chloride channel. A goat demonstrating the disease had a +47 mV shift in the channel activation, which created less open chloride channels located near the rested membrane of the skeletal muscles, which demonstrates a molecular basis-decreased chloride production in the myotonic muscles. The mutation causes a delay in the relaxation of the muscles after the goat has made an involuntary movement. After stimulation, in myotonia congenita there is an increased tendency of the muscle fibers to respond with repetitive action potentials and after discharges.Martin, A. F., Bryant, S. H., & Mandel, F. (1984). Isomyosin distribution in skeletal muscles of normal and myotonic goats. Muscle & Nerve, 7(2), 152–160. It has been shown that the increased muscle excitability is largely accounted for by the lack of chloride permeability in these fibers. Myotonia congenita is also characterized by a significant increase in the fast isomyosins in each muscle type. The muscle fibers of the myotonic goat were found to be highly (electrically) resistive, corresponding to the blocking of chloride conductance. In a study, normal goat muscle fibers could be made myotonic by blocking the chloride conductance using myotonia inducing drugs, or by substituting in an anion that is unable to pass through a semi-permeable membrane. Isolated intercostal muscle from goats with the condition was shown to be significantly different from that of normal goats in terms of the temperature dependence of the resting membrane resistance and potassium efflux.Lipicky, R. J., & Bryant, S. H. (1972). Temperature effects on cable parameters and K efflux in normal and myotonic goats. American Journal of Physiology, 222(1), 213–215. doi:10.220.33.1 These differences help to explain increases in the severity of myotonia in the whole animal that occurs upon decreasing the temperature of the involved muscles. It has been observed that there are no abnormalities in percussion responses or stiffness during the first 14 days of a newborn goat's life. The first percussion responses were observed during days 18–143, and the stiffening and/or falling begins to occur during days 20–173. Although there is no known treatment for myotonia congenita, in a study testing the effect of hydration on myotonia in goats, it was found that upon depriving goats of
water Water is an inorganic compound with the chemical formula . It is a transparent, tasteless, odorless, and Color of water, nearly colorless chemical substance. It is the main constituent of Earth's hydrosphere and the fluids of all known liv ...
, the myotonic symptoms disappeared within 3 days and returned fully within 2–3 days of water being provided. Previous studies have also reported that
taurine Taurine (), or 2-aminoethanesulfonic acid, is a naturally occurring amino sulfonic acid that is widely distributed in animal tissues. It is a major constituent of bile and can be found in the large intestine. It is named after Latin (cogna ...
, an amino sulphonic acid, when given to myotonic patients can reduce the symptoms of the condition.Conte Camerino, D.; Bryant, S.H.; Mambrini, M.; Franconi, F.; Giotti, A. (1990). "The action of taurine on muscle fibers of normal and congenitally myotonic goats". ''Pharmacological Research''. 22: 93–94. . However, it has been shown that it neither antagonizes the condition, nor prevents it.


Relationship to humans

The condition in myotonic goats is similar to a condition in humans that is known as congenital myotonia. As with goats, humans are typically not consumed by the condition and can more or less lead normal lives. Similar to goats, in humans the condition is described as a chloride channel disorder known for delayed muscle relaxation, is also caused by mutations in the skeletal muscle chloride channel gene,Colding‐Jørgensen, Eskild (2005). "Phenotypic variability in myotonia congenita". ''Muscle & Nerve''. 32 (1): 19–34. . . and can range from mild to severe. In an experiment with humans using muscle biopsy, after Periodic acid-Schiff (PAS) staining with diastase digestion, there was PAS positive material within myotonic goat fibers.Atkinson, J. B.; Swift, L. L.; Lequire, V. S. (1981-3). "Myotonia congenita. A histochemical and ultrastructural study in the goat: comparison with abnormalities found in human myotonia dystrophica". ''The American Journal of Pathology''. 102 (3): 324–335.
ISSN An International Standard Serial Number (ISSN) is an eight-digit to uniquely identify a periodical publication (periodical), such as a magazine. The ISSN is especially helpful in distinguishing between serials with the same title. ISSNs a ...
0002-9440. PMC 1903708.
PMID PubMed is an openly accessible, free database which includes primarily the MEDLINE database of references and abstracts on life sciences and biomedical topics. The United States National Library of Medicine (NLM) at the National Institutes of ...
7212017.
Myotonic goats tend to be less preferred for sustainable meat production. Meat production from goats uses maternal breeds that demonstrate high levels of fitness under non-optimal situations. Myotonic goats' maternal fitness cannot be assessed with conventional methods. To estimate their maternal fitness, the health and reproductive traits of 80 myotonic goats were compared to those of Spanish goats, Kiko goats, and Boer goats. The study measured fecal egg count (FEC) and packed cell volume (PCV). Myotonic goats tended to have a lower body mass and a lower FEC, but a greater PCV than Boer goats. Weaning rates, annual kidding rates, doe retention rates and kid crop weaned were similar in all goats except myotonic goats, which had the lowest FEC compared to other breeds. This indicates that myotonic goats are less suited for meat production under non-optimal conditions. In 2019, the myotonic goat's
conservation status The conservation status of a group of organisms (for instance, a species) indicates whether the group still exists and how likely the group is to become extinct in the near future. Many factors are taken into account when assessing conservation ...
was listed as "at risk" in the
DAD-IS DAD-IS is the acronym for the Domestic Animal Diversity Information System, a tool developed and maintained by the Food and Agriculture Organization of the United Nations as a part of its programme for management of animal genetic resources for ...
database of the
Food and Agriculture Organization of the United Nations The Food and Agriculture Organization of the United Nations; . (FAO) is a specialized agency of the United Nations that leads international efforts to defeat hunger and improve nutrition and food security. Its Latin motto, , translates t ...
. The Myotonic Goat Registry (MGR) believes it is important to maintain the breed, and that myotonic goats have other desirable traits than their "fainting" behavior. The MGR states that these traits include quiet behavior, parasite resistance, good mothering ability, and for the observation of their fainting behavior. Myotonic goats tend not to jump over fences higher than . This allows farmers to have less vertical fencing compared with other goats. The MGR also registers percentage Myotonic goats in an attempt to ensure an accurate registry. This allows for identification of purebred myotonic goats which may be used for meat, milk, or pets.


References

{{Goat breeds of Canada and the United States Meat goat breeds Conservation Priority Breeds of the Livestock Conservancy Goat breeds originating in the United States Sheep and goat diseases