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1.
The organisation of the E1alpha subunit of bovine branched-chain alpha-keto acid dehydrogenase gene was established. c DNA was cloned from Poll Shorthorn x Poll Hereford calves affected with Maple Syrup Urine Disease to identify the mutation responsible for the disease in Poll Shorthorns. Clones containing the c DNA sequences inherited from the Poll Shorthorn sire of the affected calves were identified. Paternal clones were sequenced and a cytidine to thymidine transition was found at nucleotide 1380. The mutation is predicted to substitute leucine in place of a highly conserved proline at codon 372. A polymerase chain reaction procedure was developed for detection of the 1380C-->T mutation in genomic DNA. Three Poll Shorthorn parents of affected calves and three affected Poll Shorthorn x Poll Hereford calves were heterozygous and an affected Poll Shorthorn calf was homozygous for this mutation. An improved polymerase chain reaction procedure was also devised to genotype Poll Herefords for the 248C-->T mutation. The procedures will facilitate disease prevention programs and assist in differential diagnosis of conditions in new-born calves that present with a rapid onset of progressive neurological disease and are characterised histologically by 'status spongiosus'. Maple Syrup Urine Disease (MSUD) is an autosomal recessive defect reported in humans (Danner and Elsas 1989), and in Poll Hereford (PH) and Poll Shorthorn (PS) calves (Harper et al 1986, Healy et al 1992). The clinical, biochemical and pathological manifestations of the disease are identical in the two breeds of cattle, and are characterised by the rapid onset of progressive neurological disease, leading to death within a few days of birth. The disease is caused by a deficiency of activity of the mitochondrial enzyme branched-chain alpha-keto acid dehydrogenase (BCKADH). This deficiency leads to elevated concentrations, in blood and tissues, of branched chain alpha-keto acids and their precursors, the branched chain amino acids, valine, leucine and isoleucine. BCKADH consists of four subunits E1alpha, E1beta, E2 and E3 that are encoded by separate genes, and MSUD may result from deficiency of any of the subunits. In PH s, the disease in caused by premature termination of translation, of the E1alpha subunit, that is induced by a cytidine to thymidine transition exon 2 (248C-->T), that converts the glutamine codon -6 to a stop codon (Q-6ST; Zhang et al 1990). We have shown that MSUD -affected PSxPH calves are heterozygous at the PH locus, illustrating molecular heterogeneity exists for bovine MSUD (Healy and Dennis 1994a). The fact that these crossbred calves are affected, indicates the PS, like the PH mutation, resides in the E1alpha subunit. 相似文献
2.
SUMMARY The clinical, pathological and biochemical manifestations of maple syrup urine disease (MSUD) are similar in Poll Hereford and Poll Shorthorn X Poll Hereford calves. No significant differences were observed in branched-chain amino acid concentrations in plasma, or of branched-chain keto acid dehydrogenase activity in fibroblasts, between Poll Herefords homozygous normal and heterozygous for the mutation responsible for MSUD. Haemopoietic chimerism resulted in incorrect diagnosis of the MSUD genotype in 30% of non-identical twins when blood DNA was analysed using allele-specific amplification. Hair roots are shown to be a suitable source of target DNA for genotyping Poll Hereford cattle for the MSUD mutation. Twelve of 203 (5.8%) aged Poll Hereford bulls, sampled at saleyards during the last 4 months of 1993, were found to be heterozygous for the mutation. In contrast, the mutant sequence was detected in only 1 of 150 (0.7%) 2- and 3-year-old Poll Hereford bulls offered for sale at 2 stud sales held during 1993, suggesting that the prevalence of the disease may decline over the next few years. 相似文献
3.
Healy PJ Dennis JA Windsor PA Pierce KD Schofield PA 《Australian veterinary journal》2002,80(11):695-697
OBJECTIVE: To develop a routine procedure for establishing the inherited congenital myoclonus (ICM) genotype of cattle and to obtain an estimate of the prevalence of heterozygotes for ICM and maple syrup urine disease (MSUD) in Australian Poll Herefords. DESIGN: A mismatch amplification procedure was developed to genotype for ICM. The ICM and MSUD genotypes of subjects from a 'neuraxial oedema' experimental breeding herd were investigated. Tail hair roots were used as a source of target DNA to determine the ICM and MSUD genotypes of 455 Poll Hereford bulls. RESULTS: An Acc I mismatch procedure was found to be suitable to genotype cattle for the ICM alleles using tail hair roots as the source of DNA. Based on the prevalence of heterozygotes among saleyard and sale bulls in the early 1990s, and contemporary slaughter bulls, the frequencies of the alleles responsible for ICM and MSUD were estimated to be between 0.01 and 0.02. CONCLUSION: This survey demonstrates that the mutations responsible for ICM and MSUD are present in the Australian Poll Hereford population. PCR tests could be used to advantage in differential diagnosis of neurological disease in newly born calves and in selection of Poll Hereford seed stock. 相似文献
4.
Reproductive traits and preweaning growth of progeny from young Hereford, Red Poll, Hereford X Red Poll, Red Poll X Hereford, Angus X Hereford, Angus X Charolais, Brahman X Hereford and Brahman X Angus dams were evaluated. First-calf heifers were mated with Red Angus bulls; Santa Gertrudis sires were used for each cow's second and third breeding season. Herefords, Red Polls and Hereford-Red Poll crosses were below average in percentage of calves weaned, whereas Angus-sired and Brahman-sired dams exceeded the overall mean. Angus X Charolais (P less than .10), Brahman X Hereford (P less than .01) and Brahman X Angus (P less than .10) dams weaned a higher percentage of calves than straightbred Herefords. None of these breed types differed from young Angus X Hereford females in reproductive performance. Angus X Charolais calves ranked highest in 180-d calf weight, exceeding progeny from both Hereford (P less than .01) and Angus X Hereford (P less than .10) dams. Brahman X Hereford dams weaned heavier (P less than .05) calves than Herefords, but their progeny did not differ at weaning from those reared by Angus X Herefords. Calves from Brahman X Angus dams weighed 12.7 kg less (P less than .01) than Angus X Hereford progeny. Analysis of the Hereford-Red Poll diallel showed evidence of (P less than .10) maternal heterosis in 180-d calf weight.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
5.
D O'Toole D L Montgomery L Steadman B O'Rourke W Russell J Dennis 《Journal of veterinary diagnostic investigation》2005,17(6):546-553
Various forms of status spongiosus occur in neonatal cattle, the best characterized of which is due to mutations of the branched-chain alpha-keto acid dehydrogenase complex (BCKD), resulting in bovine maple syrup urine disease (MSUD, branched-chain ketoaciduria). A distinctive neurological syndrome was identified between 1998 and 2003 in 9 calves in a 250-cow stabilized Gelbvieh-Red Angus herd. Both sexes were affected (6 heifers, 3 bulls), with a low annual incidence (3 cases in 1998; no cases in 1999; 2 cases in 2000; 2 in 2001; 1 in 2002; 1 in 2003). Affected calves were born full-term, unable to stand, and had constant whole-body tremors when stimulated. Animals remained in lateral recumbency until death or euthanasia; the longest survival time was 10 days postpartum. The principal histological change in 2 affected calves was diffuse, moderately severe bilaterally symmetrical status spongiosus with Alzheimer type II cells throughout the white matter of the brain. Myelin deficits were not evident and vacuoles were due to cleaved myelin sheaths. Neither recognized mutation of MSUD was identified in the E1a subunit of BCKD in 2 affected calves, 8 dams that gave birth to affected calves, a grand-dam of 3 affected calves, or a sire of 1 calf. Amino acid analysis of serum from 1 affected calf revealed normal concentrations of branched-chain amino acids, indicating that this disease is distinct from MSUD. The genetic and biochemical basis for the disorder, provisionally named congenital status spongiosus of Gelbvieh-cross cattle, is undetermined. The pattern of inheritance was not established. 相似文献
6.
Mature dams representing Hereford, Red Poll, F1 Hereford x Red Poll, F1 Red Poll x Hereford, F1 Angus x Hereford, F1 Angus x Charolais, F1 Brahman x Hereford and F1 Brahman x Angus breed types were evaluated. All cows were bred to Limousin sires to produce two-way or three-way-cross progeny. Mature Brahman x Hereford dams produced a higher (P less than .05) percentage of live calves than Herefords, but dam breed differences in percentage of calves weaned relative to the number of cows exposed for mating were not statistically significant. Progeny of Angus x Charolais and Red Poll dams were outstanding in weaning weight, but Hereford and Brahman-cross calves were below average. Planned comparisons showed that Angus x Charolais calves were heavier (P less than .01) at weaning than Hereford (23.0 +/- 3.8 kg) or Angus x Hereford (9.6 +/- 3.2 kg) progeny. Mature Angus x Hereford mothers weaned heavier calves than did Brahman x Herefords (7.4 +/- 3.2 kg, P less than .05) or Brahman x Angus (10.9 +/- 3.0 kg, P less than .01). Analysis of the Hereford-Red Poll diallel showed evidence of maternal heterosis in calf weaning weight (4.0 +/- 2.6 kg, P less than .05), but there was no difference in the percentage of calves weaned by crossbred vs straightbred dams.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
7.
Estimates of branched chain amino acid concentrations in plasma and, or, serum, urine, cerebrospinal fluid, formalin-fixed cerebral tissue and the associated formalin, provided evidence for a diagnosis of branched chain keto acid decarboxylase deficiency in five polled Hereford calves. The similarity of the clinical signs of dullness, recumbency and opisthotonos, and the observation of severe status spongiosus within the central nervous system, indicated that this condition had probably affected seven other newborn calves. It is suggested that this condition is analogous to branched chain keto acid decarboxylase deficiency or maple syrup urine disease of children. 相似文献
8.
The objective of this study was to determine whether F1 cows that differ in genetic potential for weight at maturity and milk yield vary in the conversion of food energy to calf weight gain. Food intakes and weight change data were recorded by pen for cows and calves from approximately 45 d postpartum. Cows assigned to the study were 7- to 9-yr-old F1s produced by top-crossing Angus, Hereford, Brown Swiss, Chianina, Gelbvieh; Maine Anjou, and Red Poll sires to either Angus or Hereford dams. Calves were sired by Simmentals. Experimental units were pens (10 to 12 cow/calf pairs); pen was replicated within breed of sire in each of 2 yr (n = 24). Calf weight gain and energy consumed by the dams differed among the F1s, as did the ratio of calf weight gain to energy consumed by the calf and cow. Angus or Hereford (35.8), Red Poll (35.7), or Maine Anjou (35.6) F1s produced more calf weight per unit of energy consumed (g/Mcal) by the cow and calf than Chianina (33.1) or Gelbvieh (33.7) F1 females; Brown Swiss cows were intermediate (34.3). Differences in food conversion efficiency exist among breed crosses. These differences seem to be associated with breed cross differences in genetic potential for milk yield and mature weight; an exception to this trend was the Maine Anjou. 相似文献
9.
C M Bailey 《Journal of animal science》1991,69(6):2379-2386
Females representing Hereford, Red Poll, F1 Hereford x Red Poll, F1 Red Poll x Hereford, F1 Angus x Hereford, F1 Angus x Charolais, F1 Brahman x Hereford, and F1 Brahman x Angus breed types were evaluated from birth until 10 yr of age. Of 308 females born alive, 35.7% died or were culled because of injury, serious illness, or reproductive failure. Breed types differed (P less than .01) in total number of mating seasons per cow and total number of progeny born and weaned. Values for lifetime total number of calves weaned were as follows: Hereford, 4.54; Red Poll, 5.45; Hereford x Red Poll, 4.45; Red Poll x Hereford, 5.49; Angus x Hereford, 5.98; Angus x Charolais, 5.57; Brahman x Hereford, 6.96; and Brahman x Angus, 6.22. Brahman crosses (P less than .01) and Angus x Charolais (P less than .10) exceeded Hereford dams in lifetime total number of calves weaned but did not differ from Angus x Herefords. Analysis of the Hereford-Red Poll diallel showed no evidence of heterosis in life span traits. Results indicate that breed type was a major source of variation in reproductive life span of beef-type females. F1 Bos indicus crosses and Angus x Herefords were outstanding in longevity. 相似文献
10.
Baird JD Wojcinski ZW Wise AP Godkin MA 《The Canadian veterinary journal. La revue veterinaire canadienne》1987,28(8):505-511
Five newborn grade horned or polled Hereford calves in Ontario exhibited the clinical signs characteristic of hereditary neuraxial edema, namely, inability to rise, and hyperesthesia with extensor spasms upon tactile or auditory stimulation. Histologically, there was widespread spongy vacuolation in both white and gray matter in the brains of all five calves, especially in the midbrain, brain stem, and cerebellum. Amino acid analysis of formalin-fixed cerebral tissue of three calves demonstrated an amino acid composition consistent with a diagnosis of branched chain keto acid decarboxylase deficiency. This condition has been recently reported in Hereford calves in Australia; it is characterized by the delayed onset of neurological signs and severe lesions of status spongiosus, and has been considered analogous to maple syrup urine disease of children. 相似文献
11.
Cerebellar abiotrophy in crossbred cattle 总被引：1，自引：0，他引：1
Cerebellar abiotrophy affected 9 of 74 calves sired by a Poll Hereford bull over 2 successive calving seasons. The disease was characterised by episodes of recumbency and ataxia, with hypermetria and wide base stance. Clinical signs commenced between birth and 8 months of age. Two calves which were affected first at 8 months of age recovered clinically 9 months later. Histological lesions were found in the cerebellar cortex of 7 calves and consisted of segmental degeneration and loss of Purkinje cells, and axonal swellings. The clinical signs and pathological findings were consistent with bovine familial convulsions and ataxia, which has not been described previously in Australia. The clinical signs were not attributable to the lesions observed in the cerebellum and an underlying electrophysiological abnormality is proposed. The aetiology of the condition is probably genetic and appears to have a multifactorial basis. 相似文献
12.
Congenital dyserythropoietic anaemia (CDA) is a heterogeneous group of rare genetic disorders that in humans is characterised by ineffective haematopoiesis with morphological abnormalities in erythroid precursor cells and secondary iron overload. In the 1990s, a syndrome of CDA with dyskeratosis and progressive alopecia was reported in Poll Hereford calves in Canada and the USA. We report the clinical and pathological findings in two Poll Hereford calves with this syndrome from separate properties in South Australia. The animals had a variably severe anaemia, associated with abnormal nucleated red blood cells in peripheral blood, and large numbers of rubricytes and metarubricytes with a characteristic nuclear ultrastructure in the bone marrow. Both calves were born with a wiry hair coat and a progressively ‘dirty‐faced’ appearance associated with hyperkeratosis and dyskeratosis (apoptosis). 相似文献
13.
Preweaning data collected at two locations (Kentucky, Louisiana) were utilized to evaluate breed-of-sire comparisons involving the Senepol breed of cattle. For the Kentucky study, calves sired by Senepol bulls were 1.3 kg heavier (P less than .05) at birth than calves sired by Hereford bulls; however, weaning weights were similar for the two sire groups. For the Louisiana study, calves sired by Longhorn bulls were 5.3 kg lighter (P less than .01) at birth, 20 kg lighter (P less than .01) at weaning and had weaning condition scores .5 unit less (P less than .01) than the average of calves sired by Red Poll and Senepol bulls. Also, heifers exposed to Longhorn bulls weaned 23 kg less (P less than .01) calf per heifer exposed than the average of heifers exposed to Red Poll and Senepol bulls. Calves sired by Red Poll bulls were 1.2 kg heavier (P less than .01) at birth and 12 kg heavier (P less than .01) at weaning than those sired by Senepol bulls; however, the Senepol-sired calves received higher (P less than .01) condition scores at weaning. Heifers exposed to Red Poll bulls weaned 20 kg more (P less than .05) calf per heifer exposed than did heifers exposed to Senepol bulls. 相似文献
14.
R E Sacco J F Baker T C Cartwright C R Long J O Sanders 《Journal of animal science》1989,67(8):1972-1979
Records of 2,449 births and 2,120 weanings of terminal-cross calves were used to characterize maternal productivity of first- and second-generation cows from a diallel of Angus, Brahman, Hereford, Holstein and Jersey when mated to third-breed sires. Third- and later-parity cows were randomly assigned after each parturition to Charolais and Red Poll bulls in multiple-sire pastures. Calves were weaned at approximately 7 mo of age; males were not castrated. A mixed model was assumed for data analysis. Effects included in the model were breed-type of dam, cow within breed-type of dam (random), breed of sire of calf, season of record, year of record, age of dam group, sex of calf and age of calf (covariate). Age of dam groups were 4- and 5-yr-olds, 6- and 7-yr-olds, 8-, 9- and 10-yr-olds, and those greater than 10 yr of age. Dependent variables were calf weight, shoulder width and hip width at birth, weaning weight, weaning height and survival to weaning. Holstein and Holstein crosses tended to produce the largest calves at birth and weaning. Among straightbred dams, the smallest calves were born to Brahman, whereas Hereford weaned the smallest calves. Brahman-Jersey dams produced the smallest calves at birth among crossbreds; Angus-Hereford cows weaned the smallest calves. Average maternal heterosis estimates for birth weight were small and non-significant. Calves of F1 crossbred dams were 17.4 kg heavier (P less than .01) and 1.70 cm taller (P less than .01) at weaning than calves of first-generation straightbred dams.(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
15.
Hereditary neuraxial oedema in a Poll Hereford herd 总被引：1，自引：0，他引：1
Cases of neuraxial oedema in a Poll Hereford herd were investigated and the pedigrees of affected calves determined. In all cases, and over no more than 3 generations, the pedigree led back to a common bull. Analysis of the herd breeding records supported an autosomal recessive mode of inheritance of the disease. Fractures of the femoral heads, acetabular cartilage and/ or bone, or both, were seen in some affected calves. This lesion was probably induced during birth or soon after, and the suggestion is made that such hip lesions could be regarded as highly suggestive of hereditary neuraxial oedmea of Herefords. The majority of calves showed clinical signs at birth, but 2 calves did not develop clinical signs until they were 2 days old. 相似文献
16.
Cardiomyopathy associated with a curly hair coat in Poll Hereford calves in Australia 总被引：1，自引：0，他引：1
Fifteen newborn Poll Hereford calves from five farms died over a three-and-a-half year period. From birth all calves had a tight, curly hair coat which was not observed on any other calves on these properties. All calves with this phenotype died before reaching six months of age. Seven of the 15 calves were noticed to have exercise intolerance, hyperpnoea and dyspnoea for one to seven days before death. The incidence of additional features of the curly hair coat phenotype is also described. Post mortem examinations conducted on the calves revealed focal, diffuse and pale fibrous streaking of the entire myocardium and vascular congestion of the livers, spleens and lungs. A primary cardiomyopathy was diagnosed on the basis of the clinical and pathological findings. Analysis of the pedigrees of seven affected calves from one farm demonstrated a common ancestor for all sires and dams of affected calves and suggested a simple autosomal recessive mode of inheritance of the syndrome as a reasonable hypothesis for future experimental testing. 相似文献
17.
Metabolizable energy for maintenance (MEm) was estimated using 123 mature cows of eight diverse breed groups. Cows in each breed group were allotted at random 1) to limit-feeding to approximate maintenance or 2) to ad libitum access to feed. The MEm values were calculated by regression of change in body energy on ME intake. The MEm values for mature Hereford, Red Poll, Hereford x Red Poll, Red Poll x Hereford, Angus x Hereford, Angus x Charolais, Brahman x Hereford, and Brahman x Angus breed types were as follows: 145, 169, 148, 149, 144, 152, 139, and 143 kcal.kg-.75.d-1, respectively. Bos indicus-cross cows ranked lowest for MEm/kg.75. Angus x Hereford cows averaged highest in terms of grams of calf weaned per mature female exposed divided by yearly MEm requirement. Hereford x Red Poll reciprocal crosses on average required 640 kcal less total daily MEm per animal than Hereford and Red Poll straightbreds. 相似文献
18.
Macroscopic, histopathologic, and histochemical investigations were made on a group of eight neonatal Angus X Hereford calves, selected from an ongoing outbreak of crooked calf disease among calving heifers. Arthrogryposis of the forelimbs was seen to varying degrees in all eight animals, and torticollis was present in six calves. Histopathology, using hematoxylin and eosin stain, did not reveal any striking or consistent lesion in the affected animals; the majority of the tissues sampled were normal. Muscle samples were processed for adenosine triphosphatase (ATPase) and NADH-tetrazolium reductase (NADH-tr) histochemistry, and the data suggest that a primary myopathy is not responsible for the congenital anomalies in the affected calves. 相似文献
19.
Three sets of blood samples were obtained from beef calves of two experimental populations and assayed for various immunological measurements. The first set of samples was taken between 24 and 48 h after birth and quantified for IgG1 concentration. A second set was taken immediately prior to vaccination for infectious bovine rhinotracheitis virus (IBRV; at an average age of 164 d) and a third set taken 60 d post-vaccination. These later samples were quantified for antibodies specific to IBRV. Level of complement C3 was also quantified in the samples taken immediately prior to vaccination. Three hundred sixty-seven calves were from four Hereford lines; three lines were previously selected for growth traits and the fourth was a randomly selected control line. There were no consistent differences in immune traits among these lines. The second group of 165 animals were Angus, Hereford and Red Poll calves. While Angus calves had a higher mean IgG1 concentration at 24 to 48 h of age than Hereford or Red Poll calves, no differences among breeds were found for the other immune traits measured. Calves from older dams (greater than 3 yr old) tended to have higher mean IgG1 concentrations, pre-vaccination IBRV antibody titers and complement C3 levels than calves from 2- and 3-yr-old cows. However, these calves had lower 60-d post-vaccination IBRV titers than calves from the younger cows (P less than .05). As pre-vaccination IBRV antibody titer increased, post-vaccination IBRV antibody titer decreased (P less than .05).(ABSTRACT TRUNCATED AT 250 WORDS) 相似文献
20.
Crossbred progeny from females, 3 yr old and older, representing Brown Swiss (B), Red Poll (R), Hereford (H) and Angus (A) maternal grandsires and maternal granddams in a diallel crossing experiment were evaluated to estimate breed mean maternal heterosis (mean hMi) for each breed, maternal heterosis for specific breed cross females (hMij), average maternal heterosis for all crosses (mean hM) and breed grandmaternal effects (gM'i) for preweaning calf traits. Estimates of mean hM were significant for birth date and weight, 200-d weight and 200-d weight/cow exposed. Even though calves with crossbred dams weighed more at birth, they did not differ from calves with straightbred dams in frequency of calving assistance. The estimates of hMij were similar for most crosses and most traits, except 200-d weight and 200-d weight/cow exposed. The largest heterotic advantage was exhibited by progeny of BH reciprocal cross females, which exceeded crossbred progeny from BB and HH females for 200-d weight/cow exposed by 35.9 kg. The smallest heterotic effect was exhibited by crossbred progeny of RA reciprocal cross females. Progeny with R maternal granddams exhibited a higher (P less than .05) live calf born and weaned percentage than progeny with H maternal granddams. Progeny with B maternal granddams were born later (P less than .05) in the calving season than progeny with R maternal granddams. Progeny with A maternal granddams exhibited a lower frequency of calving assistance than progeny with H maternal granddams, and progeny with H maternal granddams were heavier (P less than .05) at 200 d than progeny with R maternal granddams. There was a tendency for estimates of breed grandmaternal effects (gMi) to exhibit an inverse relationship with estimates of breed maternal effects (gMi). 相似文献