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Biomedical subjects

R M Goodman

Publications and source records attributed to R M Goodman.

At least 91 records · Page 5Linked to original sources
PubMed1986

New technology and its role in enhancing global food production.

The transfer in the past 3 decades of modern agricultural technology to countries of the Third World has led to a steady improvement in global food production. The results have not been evenly distributed, however, and serious problems remain. Modern biotechnology may contribute to solving some of the problems of high input costs and may also contribute to decreasing the risks associated with agriculture in developing economies. Several problems must be overcome, however. Among these are finding ways to bring the advanced technological capabilities of private companies, both large and small, to the international agricultural research network where commercial incentives are not strong or are inappropriate. Also, unless and until severe countervailing forces, such as population growth rates and deterioration of the environment, are brought under control the spread of new agricultural technology will be of little consequence in the most difficult famine-prone situations.

Agriculture↗
PubMed1985

Nucleotide sequence of bean golden mosaic virus and a model for gene regulation in geminiviruses.

We have sequenced the genome of bean golden mosaic virus, which comprises two circular single-stranded DNA molecules (2646 and 2587 nucleotides long) of mostly unique sequence. Comparison of the sequences of bean golden mosaic virus and of cassava latent virus, which share serological relationship but are very different in host range and geographical origin, shows that each virus has a unique 200-nucleotide sequence (common region) on each 2.6-kilobase molecule of its genome. The common regions of the two viruses have no sequence homology except for a short inverted repeat near the 3' end. Six open reading frames were identified that possess considerable sequence homology between the two viruses and, in bean golden mosaic virus, may encode proteins of 15.6, 19.6, 27.7, 29.7, 33.1, and 40.2 kDa. Conserved open reading frames are found in both the viral strand and the complementary strand, are approximately the same size, and are in the same orientation with respect to the common region in both viruses. We propose that temporal regulation in geminiviruses depends on the polarity of transcription and that the common region represents a replication origin and contains elements that serve to modulate gene expression.

Journal Article↗
PubMed1985

A new nail dysplasia syndrome with onychonychia and absence and/or hypoplasia of distal phalanges.

A family in which two generations have a rare and previously undescribed form of nail dysplasia with bone abnormalities is reported. This disorder is characterized by onychodystrophy, anonychia, brachydactyly of the fifth digit of the hands, and digitalization of the thumbs, with absence and/or hypoplasia of the distal phalanges of the hands and feet. The probable mode of transmission is autosomal dominant.

Abnormalities, Multiple↗
PubMed1985

Spontaneous spinal cord "injury potential" in the rat.

A marked ionic change in both the intra- and the extracellular space at the site of an acute spinal cord lesion has been reported in the literature. The present study was undertaken to measure spontaneous electrical potentials that might be associated with the previously observed ionic shifts. With the use of an impact (weight drop) model of cord injury in rats, lesions were induced at T-8. DC potentials were measured simultaneously both rostrally and caudally with respect to site of injury over a time course of 4 hours after injury. The potentials were positive with respect to lesion site, and the intensity decreased with time during 4 hours of observation. These results seem to support reported ionic shifts and migrations in injured cords and represent, to our knowledge, the first reported measurement of spontaneous injury potential in the cord of a mammal.

Animals↗
PubMed1984

A new syndrome with camptodactyly, joint contractures, facial anomalies, and skeletal defects: a case report and review of syndromes with camptodactyly.

A new camptodactyly syndrome is described in a 16-year-old Sephardic Jewish girl consisting of unusual facies with multiple eye anomalies, short stature, scoliosis, and joint contractures. Parental consanguinity is suggestive of an autosomal recessive mode of inheritance, although a new autosomal dominant mutation cannot be excluded. Fourty-four syndromes associated with camptodactyly are summarized and reviewed.

Adolescent↗
PubMed1984

Stub thumbs in Israel revisited.

A three generation family with stub thumbs and short fourth toes is reported. This combination of anomalies has been observed in the past and the question is raised whether this association could be genetically distinct from that of stub thumbs alone.

Adolescent↗
PubMed1983

Spongy degeneration of the brain in Israel: a retrospective study.

Spongy degeneration of the brain is a rare autosomal recessive disorder found predominantly in Ashkenazi Jews. In a recent review, 48 families were found to be affected with this disease. This report deals with a survey of this condition in Israel between the years 1965-1980. Eleven families with 17 affected children were ascertained. All records on these patients were reviewed and each family was visited. All the families were Jewish, comprising 7 Ashkenazi, 1 Sephardi, 2 Oriental and 1 mixed Ashkenazi-Oriental family. Parental consanguinity was noted only in the Sephardi and Oriental families, suggesting the rareness of the gene in these populations, versus its relatively more frequent occurrence among Ashkenazi Jews. Various clinical and histopathological findings observed in our study are compared to those reported in the literature.

Adult↗
PubMed1983

Altered protein glycosylation and procollagen to collagen conversion in human fibroblasts.

Skin fibroblasts, from a 9-year-old girl with an apparent heritable disorder of connective tissue, were cultured in vitro. The biosynthesis of the extracellular matrix glycoproteins, procollagens, and fibronectin was studied using radioactively labeled sugars and amino acids. Glycoproteins synthesized and secreted into the growth medium were found to be only partially glycosylated and the extracellular limited proteolytic conversion of procollagen type I to collagen was impaired, with procollagen chains accumulating in the growth medium. These biosynthetic alterations have not been previously reported in human skin fibroblasts, but similar findings have been described in tunicamycin-treated chick fibroblasts.

Biological Transport↗
PubMed1982

Upper limb involvement in the Klein-Waardenburg syndrome.

Upper limb involvement in the Klein--Waardenburg (K--W) syndrome is documented in two affected sibs and in four other previously reported patients. In addition to the key facial and auditory findings observed in the Waardenburg syndrome type I, these patients have such bilateral upper limb defects as hypoplasia of the musculoskeletal system, flexion contractures, fusion of the carpal bones, and syndactyly. The cause of the K--W syndrome is not known although there is some evidence for autosomal dominant inheritance, but further documentation is needed before this can be considered conclusive.

Abnormalities, Multiple↗
PubMed1982

Autosomal dominant inheritance of retinoschisis.

Hereditary retinoschisis affected eight members of three generations of a family. The mode of transmission and the clinical features were not compatible with findings noted in either X-chromosome-linked or autosomal recessive forms of retinoschisis. The genetic and clinical features in this family strongly supported autosomal dominant inheritance, adding to the known genetic heterogeneity for the hereditary forms of retinoschisis. The expression of the condition varied in severity, but all affected members of the family had peripheral retinoschisis and peripheral retinal degeneration. Three had maculoschisis and five had macular pigmentary changes. Electroretinographic findings were normal in six of the eight.

Adult↗
PubMed1982

Teaching human genetics in the clinical years of medical school.

A multidisciplinary approach has been presented for the teaching of clinical genetics to medical students. No set program or specific course has been put forth, but rather a continuous exposure to patients with genetic disorders has been advocated as an integral part of a medical student's learning experience during his or her years of clinical study. Four teaching aims and various approaches to implementing these aims have been discussed. They involve 1) giving the student a broad exposure to clinical genetics, 2) acquainting the student with the main clinical and laboratory procedures used in the diagnosis of genetic diseases, 3) making the student aware of the problems and complexities of genetic counseling, and 4) creating an appreciation for the continuous need for investigative studies. With the exception of genetic counseling, all of the aims could be achieved as the student rotates through the various specialties in the clinical years. However, joint teaching efforts by departments of medical genetics and other clinical departments can and should make for an enriching experience.

Curriculum↗
PubMed1982

The heterozygote female in X-linked recessive primary retinal dysplasia.

Three families with primary retinal dysplasia are reported. The ophthalmoscopical findings vary from congenital retinal folds to highly disorganized tumor-like protrusions in the vitreous cavity. The family pedigrees along with the clinical features support an X-linked recessive mode of transmission for this condition. Female carriers for this gene may show retinal fold changes. In addition some of these presumed female carriers also demonstrated changes in the stroma of their irides resulting in a gray to grayish-blue color.

Adult↗