Fish muscle hydrolysate obtained using largemouth bass Micropterus salmoides digestive enzymes improves largemouth bass performance in its larval stages

The present study utilized digestives tracts from adult largemouth bass (LMB) to hydrolyze Bighead carp muscle and obtain an optimal profile of muscle protein hydrolysates that would be easily assimilated within the primitive digestive tract of larval LMB. Specifically, muscle protein source was digested for the larva using the fully developed digestive system of the same species. The objectives of this study were: 1) to develop an optimal in vitro methodology for carp muscle hydrolysis using LMB endogenous digestive enzymes, and 2) to evaluate the effect of dietary inclusion of the carp muscle protein hydrolysate on LMB growth, survival, occurrence of skeletal deformities, and whole-body free amino acid composition. The study found that the in vitro hydrolysis method using carp intact muscle and LMB digestive tracts incubated at both acid and alkaline pH (to mimic digestive process of LMB) yielded a wide range of low molecular weight fractions (peptides), as opposed to the non-hydrolyzed muscle protein or muscle treated only with acid pH or alkaline pH without enzymes from LMB digestive tracts, which were comprised of large molecular weight fractions (polypeptides above 150 kDa). Overall, the dietary inclusion of the carp muscle hydrolysate improved growth performance of larval LMB in terms of final average weight, weight gain, DGC, SGR, and body length after 21 days of feeding compared to fish that received the diet based on non-hydrolyzed carp muscle. The study also found that hydrolysate-based feed significantly reduced skeletal deformities. The positive growth performance presented by fish in the hydrolysate-fed group possibly resulted from matching the specific requirements of the larvae with respect to their digestive organ development, levels of digestive enzymes present in the gut, and nutritional requirements.

STAT3 is critical for skeletal development and bone homeostasis by regulating osteogenesis

Skeletal deformities are typical AD-HIES manifestations, which are mainly caused by heterozygous and loss-of-function mutations in Signal transducer and activator of transcription 3 (STAT3). However, the mechanism is still unclear and the treatment strategy is limited. Herein, we reported that the mice with Stat3 deletion in osteoblasts, but not in osteoclasts, induced AD-HIES-like skeletal defects, including craniofacial malformation, osteoporosis, and spontaneous bone fracture. Mechanistic analyses revealed that STAT3 in cooperation with Msh homeobox 1(MSX1) drove osteoblast differentiation by promoting Distal-less homeobox 5(Dlx5) transcription. Furthermore, pharmacological activation of STAT3 partially rescued skeletal deformities in heterozygous knockout mice, while inhibition of STAT3 aggravated bone loss. Taken together, these data show that STAT3 is critical for modulating skeletal development and maintaining bone homeostasis through STAT3-indcued osteogenesis and suggest it may be a potential target for treatments.

Description of the molecular and clinical characteristics of the mucopolysaccharidosis type VII Iberian cohort

Background Mucopolysaccharidosis type VII (Sly syndrome) is an ultra-rare neurometabolic disorder caused by inherited deficiency of the lysosomal enzyme β-glucuronidase. Precise data regarding its epidemiology are scarce, but birth prevalence is estimated to vary from 0.02 to 0.24 per 100,000 live births. The clinical course and disease progression are widely heterogeneous, but most patients have been reported to show signs such as skeletal deformities or cognitive delay. Additionally, detection criteria are not standardized, resulting in delayed diagnosis and treatment. Methods We present a cohort of 9 patients with mucopolysaccharidosis VII diagnosed in the Iberian Peninsula, either in Spain or Portugal. The diagnostic approach, genetic studies, clinical features, evolution and treatment interventions were reviewed. Results We found that skeletal deformities, hip dysplasia, hydrops fetalis, hepatosplenomegaly, hernias, coarse features, respiratory issues, and cognitive and growth delay were the most common features identified in the cohort. In general, patients with early diagnostic confirmation who received the appropriate treatment in a timely manner presented a more favorable clinical evolution. Conclusions This case series report helps to improve understanding of this ultra-rare disease and allows to establish criteria for clinical suspicion or diagnosis, recommendations, and future directions for better management of patients with Sly syndrome.

Skeletal Deformities in Cultured Juvenile African Catfish Clarias gariepinus (Burchell, 1822)

Skeletal deformities in cultured fish are known worldwide and it had affected the survival, growth and appearance of the fish which contribute to production loss. Clarias gariepinus is known as number one farmed fish in Malaysia. C. gariepinus also effected in these deformities especially in cultured species. The objectives of this study are to identify the skeletal deformities that occur in juvenile stages and to compare the bone structure between the normal and deformed fish. A total of 50 juveniles’ fish were collected from Aquaculture Extension and Community Centre Machang, Kelantan. The juvenile was measured before undergo staining process. The fish were starved for one day then fixed in 70% ethanol for two weeks. The fish were then stained with alizarin red S for cartilage and Alcian blue for bone. The specimens were photographed in order to observe the deformities. The data showed only 13.0% juveniles have skeletal deformities, 10.9% with lordosis and 2.2% scoliosis. The cause of the deformities may be due to the heterogenous growth but other factors might also contribute to the problem. As the conclusion, the skeletal deformities observed in juvenile C. gariepinus were detected with the whole-mount staining method ranged from size 7.3 and 26.0 cm in total length. This study shows there is heterogenous growth rearing at low percentage even from skilled farm. Further study should concentrate on hidden factors that affected the deformity rate and mineralization of the juvenile fish.

Three-dimensional study of the orbit-related structures according to sex, age and skeletal deformities

Objective: This study aimed to evaluate the relations between orbit-related structures and sex, age and skeletal deformities using cone-beam computed tomography (CBCT). Methods: This retrospective study evaluated 216 consecutive CBCT scans of patients, who were divided according to: sex (male, n=105; female, n=111), age (A1: 18-32 years, n=71; A2: 33-47 years, n=78; A3: 48-62 years, n=67), and skeletal deformities (Class I, n=70; Class II, n=75; Class III, n=71). The supraorbital foramen (SOF) location, volume of orbit, optic canal (OC) and infraorbital canal (IOC) were evaluated. Results were analyzed using the Gamma model test. The Tukey-Kramer post-hoc test was used to compare the variables with three factors (p<0.05). Results: The IOC volume showed higher values for male, A3 and class I patients. The SOF location and the orbital volume also showed higher values for male patients. Regarding the volume of CO, it showed higher values ​​for male and class I patients. Conclusions: According to our results, sex has been shown to have a significant influence on orbit-related structures. Age and skeletal deformities also influenced the volume of IOC and OC. These results eventually help the clinical practice, being useful for orbital reconstruction surgeries, anthropological studies, gender identification and identification of susceptibility to pathological conditions related to sexual dimorphism.

Skeletal Deformities in Osterix-Cre;Tgfbr2f/f Mice May Cause Postnatal Death

Transforming growth factor β (TGFβ) signaling plays an important role in skeletal development. We previously demonstrated that the loss of TGFβ receptor II (Tgfbr2) in Osterix-Cre-expressing mesenchyme results in defects in bones and teeth due to reduced proliferation and differentiation in pre-osteoblasts and pre-odontoblasts. These Osterix-Cre;Tgfbr2f/f mice typically die within approximately four weeks for unknown reasons. To investigate the cause of death, we performed extensive pathological analysis on Osterix-Cre- (Cre-), Osterix-Cre+;Tgfbr2f/wt (HET), and Osterix-Cre+;Tgfbr2f/f (CKO) mice. We also crossed Osterix-Cre mice with the ROSA26mTmG reporter line to identify potential off-target Cre expression. The findings recapitulated published skeletal and tooth abnormalities and revealed previously unreported osteochondral dysplasia throughout both the appendicular and axial skeletons in the CKO mice, including the calvaria. Alterations to the nasal area and teeth suggest a potentially reduced capacity to sense and process food, while off-target Cre expression in the gastrointestinal tract may indicate an inability to absorb nutrients. Additionally, altered nasal passages and unexplained changes in diaphragmatic muscle support the possibility of hypoxia. We conclude that these mice likely died due to a combination of breathing difficulties, malnutrition, and starvation resulting primarily from skeletal deformities that decreased their ability to sense, gather, and process food.

Incidence of skeletal deformities in induced triploid rainbow trout Oncorhynchus mykiss (Walbaum, 1792)

Due to the cytogenetic incompatibility, triploid fish are usually infertile and are not affected by a decline in growth, survival and meat quality, which accompanies the process of sexual maturation in diploid specimens. Thus, artificial triploidization has been proposed for fish production in the case of species with early sexual maturation, such as rainbow trout. However, the use of this technique is limited by increased ratios of skeletal deformities observed in triploid specimens. The main objective of this research was to compare the proportion and variety of body abnormalities in diploid and triploid 14-month-old rainbow trout from commercial stocks, using external body shape examination, radiography and whole-mount skeletal staining. Individuals with externally observed body deformities (scoliosis, humpback, shortened tail and jaw deformities) accounted for 0.45% of the diploid stock and 3.83% of the triploid stock. X-rays and whole-mount skeletal staining of deformed individuals showed spine deformities, including compressions and fusions of vertebrae. Abnormalities observed in diploid and triploid rainbow trout examined during this study were non-lethal, however, they may negatively affect the condition of fish. Fish with skeletal deformities are not aesthetically pleasing, thus an increased ratio of such deformations in fish produced for commercial purposes may result in real economic losses.

The Innermore Ossein- Enchondroma

Preface Frequently, benign bone tumours are an incidental discovery wherein clinical symptoms are contingent to tumour location and magnitude and manifest as localized pain, swelling, skeletal deformities or pathologic fracture. Radiographic imaging is a cogent methodology of discerning the neoplasms.