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Applied Microbiology and Biotechnology May 2013Early biotechnology (BT) had its roots in fascinating discoveries, such as yeast as living matter being responsible for the fermentation of beer and wine. Serious... (Review)
Review
Early biotechnology (BT) had its roots in fascinating discoveries, such as yeast as living matter being responsible for the fermentation of beer and wine. Serious controversies arose between vitalists and chemists, resulting in the reversal of theories and paradigms, but prompting continuing research and progress. Pasteur's work led to the establishment of the science of microbiology by developing pure monoculture in sterile medium, and together with the work of Robert Koch to the recognition that a single pathogenic organism is the causative agent for a particular disease. Pasteur also achieved innovations for industrial processes of high economic relevance, including beer, wine and alcohol. Several decades later Buchner, disproved the hypothesis that processes in living cells required a metaphysical 'vis vitalis' in addition to pure chemical laws. Enzymes were shown to be the chemical basis of bioconversions. Studies on the formation of products in microbial fermentations, resulted in the manufacture of citric acid, and chemical components required for explosives particularly in war time, acetone and butanol, and further products through fermentation. The requirements for penicillin during the Second World War lead to the industrial manufacture of penicillin, and to the era of antibiotics with further antibiotics, like streptomycin, becoming available. This was followed by a new class of high value-added products, mainly secondary metabolites, e.g. steroids obtained by biotransformation. By the mid-twentieth century, biotechnology was becoming an accepted specialty with courses being established in the life sciences departments of several universities. Starting in the 1970s and 1980s, BT gained the attention of governmental agencies in Germany, the UK, Japan, the USA, and others as a field of innovative potential and economic growth, leading to expansion of the field. Basic research in Biochemistry and Molecular Biology dramatically widened the field of life sciences and at the same time unified them considerably by the study of genes and their relatedness throughout the evolutionary process. The scope of accessible products and services expanded significantly. Economic input accelerated research and development, by encouraging and financing the development of new methods, tools, machines and the foundation of new companies. The discipline of 'New Biotechnology' became one of the lead sciences. Although biotechnology has historical roots, it continues to influence diverse industrial fields of activity, including food, feed and other commodities, for example polymer manufacture, biofuels and energy production, providing services such as environmental protection, and the development and production of many of the most effective drugs. The understanding of biology down to the molecular level opens the way to create novel products and efficient environmentally acceptable methods for their production.
Topics: Biotechnology; History, 19th Century; Industrial Microbiology
PubMed: 23504077
DOI: 10.1007/s00253-013-4768-2 -
Archives Francaises de Pediatrie Dec 1978Fibrochondrogenesis is a lethal form of dwarfism similar to thanatophoric dwarfism. It is distinguished radiologically by the widening of the metaphyses of the long...
Fibrochondrogenesis is a lethal form of dwarfism similar to thanatophoric dwarfism. It is distinguished radiologically by the widening of the metaphyses of the long bones, and, on lateral x-ray of the spine, by a median fissure of the body of the vertebra without any loss of vertebral height. The condition is inherited an autosomal recessive. A study of growing cartilage confirms that this disease is a distinct entity as there is fibrosis of the cartilage which is never present in thanatophoric dwarfism or in the different forms of achondrogenesis.
Topics: Bone Diseases, Developmental; Cartilage; Diagnosis, Differential; Dwarfism; Humans; Infant, Newborn; Proteoglycans
PubMed: 749746
DOI: No ID Found -
Journal of Clinical Orthopaedics and... Mar 2024Proximal tibia depression fracture often occur isolated or in conjunction with complex fracture presentations and elevation of such depression is required to retard...
Proximal tibia depression fracture often occur isolated or in conjunction with complex fracture presentations and elevation of such depression is required to retard arthritis in long term. Conventional open reduction by sub meniscal approach has many percutaneous alternatives from arthroscopy assisted reduction to balloon tibioplasty. Few authors even reported usage of PCL jig and percutaneous pins to elevate, while the primary author has previously described an instrument to elevate the depressed fragment percutaneously. With the shortcomings of the same instrument, authors have designed modifications in the same to address anterior and posterior extensions of depression without widening the metaphyseal window. In this article, we describe the size and concept of the modified design and its efficacy to address depression injuries.
PubMed: 38450412
DOI: 10.1016/j.jcot.2024.102383 -
Indian Journal of Orthopaedics Jul 2021Depressed lateral tibial plateau fractures with a large central or posterior fragment can be seen in isolation or association with complex proximal tibia fractures....
Depressed lateral tibial plateau fractures with a large central or posterior fragment can be seen in isolation or association with complex proximal tibia fractures. Conventionally elevation of the large depressed fragment is done by bone tamp through a medial metaphyseal window in isolated fractures, or the fractured window in associated complex fracture scenarios. Though various instruments have been devised for this purpose, reaching the posterior aspect of lateral condyle through the medial metaphyseal window is not always easy, considering the difficulty in aiming and trajectory. Excessive maneuvering can result in the widening of the medial metaphyseal window, leaves a large metaphyseal void, intraarticular penetration of elevating device, and comminution of the depressed fragment. Described herein is an alternate percutaneous technique for effective reduction of selected lateral tibial plateau depression fractures using Steinmann pin. Twenty- one patients with at least 1-year follow-up with successful outcomes have benefitted from this reduction technical tip thus far.
PubMed: 34306563
DOI: 10.1007/s43465-021-00391-9 -
American Journal of Medical Genetics Aug 1992The Weissenbacher-Zweymüller syndrome (WZS) is defined as congenital neonatal rhizomelic dwarfism with metaphyseal widening of the long bones and vertebral coronal... (Review)
Review
The Weissenbacher-Zweymüller syndrome (WZS) is defined as congenital neonatal rhizomelic dwarfism with metaphyseal widening of the long bones and vertebral coronal clefts. Catch-up growth after 2-3 years is one of the striking manifestations. It is generally thought that WZS is a neonatal expression of the Stickler syndrome, even though in the latter, myopia, retinal detachment and a progressive metaphyseal dysplasia are characteristics that are not found in WZS. A critical analysis of all published patients with WZS in addition to 5 patients in 3 new families, shows that the WZS is a distinct syndrome of delayed skeletal maturation, different from the Stickler syndrome, and inherited as an autosomal recessive trait. The recognition of its unique characteristics has important implications in genetic counseling.
Topics: Bone Diseases, Developmental; Child; Child, Preschool; Dwarfism; Female; Genes, Recessive; Humans; Male; Phenotype; Syndrome
PubMed: 1415350
DOI: 10.1002/ajmg.1320430616 -
The British Journal of Radiology Jun 1979Pyle disease is a rare genetic skeletal disorder which is conventionally classified with craniotubular dysplasias. The radiographic manifestations in three affected...
Pyle disease is a rare genetic skeletal disorder which is conventionally classified with craniotubular dysplasias. The radiographic manifestations in three affected adults included widening of the metaphyseal portions of the long bones which extended through a major portion of the diaphyses, with cortical thinning and mild cranial sclerosis. The femora presented the characteristic Erlenmeyer flask configuration. Pyle disease is clinically, radiographically and genetically distinct from craniometaphyseal dysplasia, a relatively common condition with which it has been confused.
Topics: Adult; Bone Diseases, Developmental; Diagnosis, Differential; Female; Femur; Humans; Male; Middle Aged; Osteosclerosis; Radiography; Skull
PubMed: 465917
DOI: 10.1259/0007-1285-52-618-431 -
Journal of Veterinary Diagnostic... Sep 2021Rickets is a metabolic bone disease associated with failure of endochondral ossification and impaired osteoid mineralization in growing animals. As a consequence,... (Review)
Review
Rickets is a metabolic bone disease associated with failure of endochondral ossification and impaired osteoid mineralization in growing animals. As a consequence, affected individuals can develop gross and microscopic bone malformations. The most common causes of rickets in domestic species include vitamin D and phosphorus deficiency. Rickets has been described in multiple species; however, comprehensive postmortem characterizations with confirmatory histopathology in equids have not been published. A 6-mo-old, Thoroughbred-cross foal was diagnosed with rickets based on gross autopsy findings and microscopic examination of the ribs and long bones. Grossly, all costochondral junctions of the ribs were enlarged with a "rachitic rosary" appearance, and there were multiple fracture calluses in the rib bodies. Epiphyses and metaphyses of the long bones appeared widened on sagittal section, and their physes were irregularly thickened. Histologically, there were poorly organized columns of hypertrophic chondrocytes within the physes of affected bones, islands of chondrocytes embedded within the primary and secondary spongiosa, and faintly eosinophilic seams of poorly mineralized osteoid within the bone trabeculae. Areas of focally increased osteoclastic activity were observed in some of the sections, perhaps pointing to a more complex metabolic bone disease in a growing animal. Low serum concentrations of calcium and 25-hydroxyvitamin D were detected in an antemortem sample. The pathogenesis of these imbalances was not definitively established, but lack of sunlight exposure, low concentration of vitamin D precursors in the diet (perhaps secondary to malnutrition), or both, were suspected; a genetic basis cannot be ruled out.
Topics: Animals; Bone and Bones; Calcium; Horse Diseases; Horses; Rickets
PubMed: 34160312
DOI: 10.1177/10406387211025232 -
Journal of Bone and Mineral Research :... Nov 2010Dysosteosclerosis (DSS), an extremely rare dense bone disease, features short stature and fractures and sometimes optic atrophy, cranial nerve palsy, developmental... (Review)
Review
Dysosteosclerosis (DSS), an extremely rare dense bone disease, features short stature and fractures and sometimes optic atrophy, cranial nerve palsy, developmental delay, and failure of tooth eruption in infancy or early childhood consistent with osteopetrosis (OPT). Bone histology during childhood shows unresorbed primary spongiosa from deficient osteoclast action. Additionally, there is remarkable progressive flattening of all vertebrae and, by adolescence, paradoxical metaphyseal osteopenia with thin cortical bone. Reports of consanguinity indicate autosomal recessive inheritance, yet more affected males than females suggest X-linked recessive inheritance. We investigated a nonconsanguineous girl with DSS. Osteosclerosis was discovered at age 7 months. Our studies, spanning ages 11 to 44 months, showed weight at approximately 50th percentile, and length diminishing from approximately 30th percentile to -2.3 SD. Head circumference was +4 SD. The patient had frontal bossing, blue sclera, normal teeth, genu valgum, and unremarkable joints. Radiographs showed orbital and facial sclerosis, basilar thickening, bone-in-bone appearance of the pelvis, sclerotic long bone ends, and fractures of ribs and extremities. Progressive metaphyseal widening occurred as vertebrae changed from ovoid to flattened and became beaked anteriorly. A hemogram was normal. Consistent with OPT, serum parathyroid hormone (PTH) concentrations reflected dietary calcium levels. Serum bone alkaline phosphatase, osteocalcin, and TRACP-5b were subnormal. The iliac crest contained excessive primary spongiosa and no osteoclasts. No mutations were identified in the splice sites or exons for the genes encoding chloride channel 7, T-cell immune regulator 1, OPT-associated transmembrane protein 1, and monocyte colony-stimulating factor (M-CSF) and its receptor C-FMS, ANKH, OPG, RANK, and RANKL. Genomic copy-number microarray was unrevealing. Hence, DSS is a distinctive OPT of unknown etiology featuring osteoclast deficiency during early childhood. How osteopenia follows is an enigma of human skeletal pathobiology.
Topics: Adult; Bone and Bones; Child, Preschool; DNA Mutational Analysis; Diagnosis, Differential; Family; Female; Humans; Infant; Infant, Newborn; Osteoclasts; Osteopetrosis; Osteosclerosis; Pregnancy; Radiography, Thoracic; Tomography, X-Ray Computed
PubMed: 20499338
DOI: 10.1002/jbmr.131 -
Skeletal Radiology Apr 2000Metaphyseal dysplasia, type Jansen (JMD), is a rare skeletal dysplasia with characteristic radiographic abnormalities. Of the various types of metaphyseal dysplasia, JMD...
Metaphyseal dysplasia, type Jansen (JMD), is a rare skeletal dysplasia with characteristic radiographic abnormalities. Of the various types of metaphyseal dysplasia, JMD shows the most severe alteration in metaphyseal architecture. All of the long tubular bones, including those of the hands and feet, show metaphyseal irregularity with a fragmented appearance and slight widening. The adjacent physes are abnormally widened, while the epiphyses tend to be slightly enlarged, rounded but otherwise normal. The spine in infancy and childhood usually appears normal. This report describes a young girl with metaphyseal changes typical of JMD except for the hands and feet, which appeared normal. She also showed very unusual abnormalities of the spine. This appears, therefore, to represent a unique osteochondrodysplasia for which we propose the term spondylometaphyseal dysplasia, type Jansen.
Topics: Abnormalities, Multiple; Female; Humans; Infant, Newborn; Osteochondrodysplasias; Radiography
PubMed: 10855475
DOI: 10.1007/s002560050601 -
American Journal of Medical Genetics Dec 1998We report on a previously undescribed form of lethal osteosclerotic skeletal dysplasia in sibs from nonconsanguineous parents. Radiographic findings included increased...
We report on a previously undescribed form of lethal osteosclerotic skeletal dysplasia in sibs from nonconsanguineous parents. Radiographic findings included increased density in the base of the skull, clavicles, vertebrae, ribs, and the metaphyseal regions of the long bones. There was midface hypoplasia, a large anterior fontanel, micrognathia, and hypoplastic, wafer-thin vertebrae. The clavicles, ribs, metacarpals, metatarsals, and phalanges were especially thickened and widened. The long bones were shortened with flared metaphyses. Chondroosseous morphology of resting cartilage and growth plate was relatively normal, but there was hypercellular cortical and trabecular bone, and marrow fibrosis. Ultrastructurally, the resting chondrocytes, osteoblasts, and nonhematopoietic marrow cells had dilated rough endoplasmic reticulum (inclusion bodies). The radiographic and morphologic characteristics in this case are unique and differ from those seen in other previously reported lethal osteosclerotic skeletal dysplasias.
Topics: Abortion, Induced; Adolescent; Adult; Bone Diseases, Developmental; Bone and Bones; Family Health; Fatal Outcome; Female; Fetal Diseases; Fetus; Humans; Inclusion Bodies; Infant, Newborn; Male; Osteochondrodysplasias; Osteosclerosis; Pregnancy
PubMed: 9856576
DOI: 10.1002/(sici)1096-8628(19981204)80:4<423::aid-ajmg23>3.0.co;2-n