Aging-related satellite cell differentiation defect  occurs prematurely after Ski-induced muscle hypertrophy

To investigate the cause of skeletal muscle weakening during aging we examined the sequence of cellular changes in murine muscles. Satellite cells isolated from single muscle fibers terminally differentiate progressively less well with increasing age of donor. This change is detected before decline in satellite cell numbers and all histological changes examined here. In MSVski transgenic mice, which show type IIb fiber hypertrophy, initial muscle weakness is followed by muscle degeneration in the first year of life. This degeneration is accompanied by a spectrum of changes typical of normal muscle aging and a more marked decline in satellite cell differentiation efficiency. On a myoD-null genetic background, in which satellite cell differentiation is defective, the MSVski muscle phenotype is aggravated. This suggests that, on a wild-type genetic background, satellite cells are capable of repairing MSVski fibers and preserving muscle integrity in early life. We propose that decline in myogenic cell differentiation efficiency is an early event in aging-related loss of muscle function, both in normal aging and in some late-onset muscle degenerative conditions.

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Young children with Noonan syndrome: evaluation of feeding problems

European Journal of Pediatrics ◽

10.1007/s00431-020-03664-x ◽

2020 ◽

Vol 179 (11) ◽

pp. 1683-1688

Author(s):

Jos M. T. Draaisma ◽

Joris Drossaers ◽

Lenie van den Engel-Hoek ◽

Erika Leenders ◽

Joyce Geelen

Keyword(s):

Young Children ◽

Noonan Syndrome ◽

Early Onset ◽

Late Onset ◽

Tube Feeding ◽

Genetic Mutation ◽

First Year ◽

Feeding Problems ◽

Genetic Syndrome ◽

First Year Of Life

Abstract Noonan syndrome (NS) is a common genetic syndrome with a high variety in phenotype. Even though genetic testing is possible, NS is still a clinical diagnosis. Feeding problems are often present in infancy. We investigated the feeding status of 108 patients with clinically and genetically confirmed NS. Only patients with a documented feeding status before the age of 6 were included. A distinction was made between patients with early onset feeding problems (< 1 year) and children with late onset feeding problems (> 1 year). Seventy-one of 108 patients had feeding problems, of which 40 patients required tube feeding. Children with a genetic mutation other than PTPN11 and SOS1 had significantly more feeding problems in the first year. Fifty-two of all 108 patients experienced early onset feeding problems, of which 33 required tube feeding. A strong decrease in prevalence of feeding problems was found after the first year of life. Fifteen children developed feeding problems later in life, of which 7 required tube feeding. Conclusion: Feeding problems occur frequently in children with NS, especially in children with NS based on genetic mutations other than PTPN11 and SOS1. Feeding problems develop most often in infancy and decrease with age. What is Known:• Young children with Noonan syndrome may have transient feeding problems.• Most of them will need tube feeding. What is New:• This is the first study of feeding problems in patients with clinically and genetically proven Noonan syndrome.• Feeding problems most often develop in infancy and resolve between the age of 1 and 2.

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Molecular Regulation and Rejuvenation of Muscle Stem (Satellite) Cell Aging

The Indonesian Biomedical Journal ◽

10.18585/inabj.v7i2.73 ◽

2015 ◽

Vol 7 (2) ◽

pp. 73

Author(s):

Anna Meiliana ◽

Nurrani Mustika Dewi ◽

Andi Wijaya

Keyword(s):

Skeletal Muscle ◽

Stem Cells ◽

Stem Cell ◽

Satellite Cell ◽

Muscle Mass ◽

Satellite Cells ◽

Cell Function ◽

Muscle Stem Cell ◽

Muscle Aging ◽

Age Related

BACKGROUND: Age-related muscle loss leads to lack of muscle strength, resulting in reduced posture and mobility and an increased risk of falls, all of which contribute to a decrease in quality of life. Skeletal muscle regeneration is a complex process, which is not yet completely understood.CONTENT: Skeletal muscle undergoes a progressive age-related loss in mass and function. Preservation of muscle mass depends in part on satellite cells, the resident stem cells of skeletal muscle. Reduced satellite cell function may contribute to the age-associated decrease in muscle mass. Recent studies have delineated that the aging process in organ stem cells is largely caused by age-specific changes in the differentiated niches, and that regenerative outcomes often depend on the age of the niche, rather than on stem cell age. It is likely that epigenetic states will be better define such key satellite cell features as prolonged quiescence and lineage fidelity. It is also likely that DNA and histone modifications will underlie many of the changes in aged satellite cells that account for age-related declines in functionality and rejuvenation through exposure to the systemic environment.SUMMARY: Skeletal muscle aging results in a gradual loss of skeletal muscle mass, skeletal muscle function and regenerative capacity, which can lead to sarcopenia and increased mortality. Although the mechanisms underlying sarcopenia remain unclear, the skeletal muscle stem cell, or satellite cell, is required for muscle regeneration. Decreased muscle stem cell function in aging has long been shown to depend on altered environmental cues, whereas the contribution of intrinsic mechanisms remained less clear. Signals in the aged niche were shown to cause permanent defects in the ability of satellite cells to return to quiescence, ultimately also impairing the maintenance of self-renewing satellite cells. Therefore, only anti-aging strategies taking both factors, the stem cell niche and the stem cells per se, into consideration may ultimately be successful.KEYWORDS: satellite cell, muscle, aging, niche, regenerations

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Assessing the association between bronchiolitis in infancy and recurrent wheeze: a whole English birth cohort case–control study

Thorax ◽

10.1136/thoraxjnl-2018-212203 ◽

2019 ◽

Vol 74 (5) ◽

pp. 503-505 ◽

Cited By ~ 3

Author(s):

Robin Marlow ◽

Adam Finn ◽

John Henderson

Keyword(s):

Birth Cohort ◽

Case Control Study ◽

Late Onset ◽

Case Control ◽

Hospital Episode Statistics ◽

First Year ◽

Recurrent Wheeze ◽

Syncytial Virus ◽

First Year Of Life ◽

Control Study

The precise association between bronchiolitis and predisposition to childhood wheeze is unclear. We assessed bronchiolitis aetiology and later wheeze phenotypes in the entire 2007 English birth cohort. All infants admitted to hospital in England during their first year of life with bronchiolitis or urinary tract infection (UTI) were followed using Hospital Episode Statistics to determine risk and characteristics of wheeze admission over the subsequent 8 years. In our cohort of 21 272 children compared with UTI, the risk of wheeze admission was higher with previous bronchiolitis (risk ratio (RR) 2.4), even higher in those with non-respiratory syncytial virus bronchiolitis (RR 3.1) and persisted into late-onset wheeze (RR 1.7).

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Genome-wide identification of enhancers and transcription factors regulating the myogenic differentiation of bovine satellite cells

BMC Genomics ◽

10.1186/s12864-021-08224-7 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

Pengcheng Lyu ◽

Robert E. Settlage ◽

Honglin Jiang

Keyword(s):

Gene Expression ◽

Skeletal Muscle ◽

Transcription Factors ◽

Cell Differentiation ◽

Satellite Cell ◽

Transcription Start Site ◽

Satellite Cells ◽

Binding Sites ◽

Start Site ◽

Transcription Start

Abstract Background Satellite cells are the myogenic precursor cells in adult skeletal muscle. The objective of this study was to identify enhancers and transcription factors that regulate gene expression during the differentiation of bovine satellite cells into myotubes. Results Chromatin immunoprecipitation followed by deep sequencing (ChIP-seq) was performed to identify genomic regions where lysine 27 of H3 histone is acetylated (H3K27ac), i.e., active enhancers, from bovine satellite cells before and during differentiation into myotubes. A total of 19,027 and 47,669 H3K27ac-marked enhancers were consistently identified from two biological replicates of before- and during-differentiation bovine satellite cells, respectively. Of these enhancers, 5882 were specific to before-differentiation, 35,723 to during-differentiation, and 13,199 common to before- and during-differentiation bovine satellite cells. Whereas most of the before- or during-differentiation-specific H3K27ac-marked enhancers were located distally to the transcription start site, the enhancers common to before- and during-differentiation were located both distally and proximally to the transcription start site. The three sets of H3K27ac-marked enhancers were associated with functionally different genes and enriched with different transcription factor binding sites. Specifically, many of the H3K27ac-marked enhancers specific to during-differentiation bovine satellite cells were associated with genes involved in muscle structure and development, and were enriched with binding sites for the MyoD, AP-1, KLF, TEAD, and MEF2 families of transcription factors. A positive role was validated for Fos and FosB, two AP-1 family transcription factors, in the differentiation of bovine satellite cells into myotubes by siRNA-mediated knockdown. Conclusions Tens of thousands of H3K27ac-marked active enhancers have been identified from bovine satellite cells before or during differentiation. These enhancers contain binding sites not only for transcription factors whose role in satellite cell differentiation is well known but also for transcription factors whose role in satellite cell differentiation is unknown. These enhancers and transcription factors are valuable resources for understanding the complex mechanism that mediates gene expression during satellite cell differentiation. Because satellite cell differentiation is a key step in skeletal muscle growth, the enhancers, the transcription factors, and their target genes identified in this study are also valuable resources for identifying and interpreting skeletal muscle trait-associated DNA variants in cattle.

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Infection

10.1093/med/9780198703952.003.0012 ◽

2017 ◽

Author(s):

Grenville Fox ◽

Nicholas Hoque ◽

Timothy Watts

Keyword(s):

Herpes Simplex ◽

Hepatitis B ◽

Late Onset ◽

Group B Streptococcus ◽

Congenital Infection ◽

First Year ◽

Fungal Sepsis ◽

Group B ◽

First Year Of Life ◽

Oriented Approach

This chapter provides a problem-oriented approach to investigation and treatment of early and late onset neonatal bacterial infection, including group B streptococcus, meningitis, urine infection, conjunctivitis, umbilical sepsis, osteomyelitis, and septic arthritis. In addition, the prevention and management of congenital infection is covered, including hepatitis B and C, HIV, syphilis, CMV, toxoplasma, rubella, herpes simplex, and chickenpox. Other topics covered are infection control (including MRSA), fungal sepsis, TB, and an overview of immunizations in the first year of life.

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Nephropathic Cystinosis in Adults

10.1093/med/9780199972135.003.0060 ◽

2016 ◽

Author(s):

Aude Servais

Keyword(s):

Age Of Onset ◽

Late Onset ◽

First Year ◽

Nephropathic Cystinosis ◽

Lysosomal Storage ◽

Crystal Deposition ◽

Proximal Tubulopathy ◽

Stage Renal Disease ◽

End Stage ◽

First Year Of Life

Cystinosis is an autosomal recessive lysosomal storage disorder caused by a defect in the carrier-mediated system that normally transports cystine out of lysosomes. As a consequence, tissues accumulate variable amounts of the disulphide amino acid cystine. Three overlapping clinical phenotypes are recognized, varying in severity and age of onset. The most severe, the infantile nephropathic form (MIM 219800), appears in the first year of life. The late-onset form (MIM 219900) is also nephropathic, while ocular, non-nephropathic cystinosis manifests largely with corneal crystal deposition (MIM 219750). Infantile cystinosis is the most common form. Affected children develop renal proximal tubulopathy at 6 to 12 months of age. In the absence of treatment, renal failure occurs, with progression to end-stage renal disease (ESRD). Cystine crystal deposition in the cornea leads to photophobia and continuous widespread cystine accumulation eventually leads to rickets, retinal, endocrinological (hypothyroidism and impaired glucose tolerance), hepatic, gastrointestinal, muscular, and neurological abnormalities.

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192 Identification of Fos and FosB as Transcriptional Regulators of Bovine Satellite Cell Differentiation

Journal of Animal Science ◽

10.1093/jas/skab235.186 ◽

2021 ◽

Vol 99 (Supplement_3) ◽

pp. 103-103

Author(s):

Pengcheng Lyu ◽

Robert Settlage ◽

Honglin Jiang

Keyword(s):

Cell Differentiation ◽

Satellite Cell ◽

Satellite Cells ◽

Chromatin Immunoprecipitation ◽

Enrichment Analysis ◽

Transcriptional Regulators ◽

Adult Skeletal Muscle ◽

Muscle Cell Differentiation ◽

Before And After ◽

Myogenic Precursor

Abstract Transcription factors (TFs) are key regulators of gene expression during cell differentiation. Four TFs including Myf5, MyoD, MyoG and Myf6 have been identified as key myogenic regulatory factors (MYFs) that regulate gene transcription during myogenesis. Satellite cells (SCs) are the myogenic precursor cells in adult skeletal muscle. The objective of this study was to identify additional TFs that control the differentiation of bovine satellite cells. Bovine satellite cells (bSCs) were isolated from 4 crossbred steers and were initially cultured in growth medium for 12 days to expand and then in differentiation medium for 48 hours to differentiate. Chromatin immunoprecipitation followed by sequencing (ChIP-seq) was performed to identify chromatin regions marked with acetylation of histone H3 on lysine 27 (H3K27ac). This ChIP-seq analysis revealed 3,348 and 38,800 H3K27ac-associated chromatin regions in bSCs before and after differentiation, respectively. A motif enrichment analysis of the H3K27ac-marked chromatin regions from the differentiated bSCs indicated the enrichment of binding sites for the 4 MYFs and many other TFs including Fos and FosB. RNA-sequencing revealed the upregulation of Fos and FosB mRNAs in bSCs from growth to differentiation. To verify the roles of Fos and FosB in bSC differentiation, their expressions in bSCs were reduced by siRNA-induced knockdown. Based on qRT-PCR analyses, expressions of MYH2, MYH3, MYOG, and CKM mRNAs, which were selected as markers of muscle cell differentiation, were increased (P < 0.05) in bSCs from growth to differentiation, but the increases in at least three of them were reversed (P < 0.05) by Fos or FosB knockdown. Taken together, these results establish Fos and FosB as transcriptional regulators of bovine satellite cell differentiation.

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microRNA-1 and microRNA-206 regulate skeletal muscle satellite cell proliferation and differentiation by repressing Pax7

The Journal of Cell Biology ◽

10.1083/jcb.200911036 ◽

2010 ◽

Vol 190 (5) ◽

pp. 867-879 ◽

Cited By ~ 352

Author(s):

Jian-Fu Chen ◽

Yazhong Tao ◽

Juan Li ◽

Zhongliang Deng ◽

Zhen Yan ◽

...

Keyword(s):

Skeletal Muscle ◽

Cell Proliferation ◽

Cell Differentiation ◽

Satellite Cell ◽

Satellite Cells ◽

Adult Stem Cells ◽

Myoblast Differentiation ◽

Proliferative Potential ◽

Satellite Cell Proliferation

Skeletal muscle satellite cells are adult stem cells responsible for postnatal skeletal muscle growth and regeneration. Paired-box transcription factor Pax7 plays a central role in satellite cell survival, self-renewal, and proliferation. However, how Pax7 is regulated during the transition from proliferating satellite cells to differentiating myogenic progenitor cells is largely unknown. In this study, we find that miR-1 and miR-206 are sharply up-regulated during satellite cell differentiation and down-regulated after muscle injury. We show that miR-1 and miR-206 facilitate satellite cell differentiation by restricting their proliferative potential. We identify Pax7 as one of the direct regulatory targets of miR-1 and miR-206. Inhibition of miR-1 and miR-206 substantially enhances satellite cell proliferation and increases Pax7 protein level in vivo. Conversely, sustained Pax7 expression as a result of the loss of miR-1 and miR-206 repression elements at its 3′ untranslated region significantly inhibits myoblast differentiation. Therefore, our experiments suggest that microRNAs participate in a regulatory circuit that allows rapid gene program transitions from proliferation to differentiation.

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Vocalization frequency as a prognostic marker of language development following early cochlear implantation

Audiology Research ◽

10.4081/audiores.2019.217 ◽

2019 ◽

Vol 9 (1) ◽

Author(s):

Paris Binos ◽

Elena Loizou

Keyword(s):

Language Development ◽

Cochlear Implantation ◽

Sound Production ◽

Late Onset ◽

First Year ◽

Substantial Impact ◽

Linguistic Performance ◽

First Year Of Life ◽

Frequency Current ◽

Implantation Success

Despite their potential significance for later linguistic outcomes, early aspects of vocalization had been seriously undervalued in the past, and thus, minimally investigated until relatively recently. The present article sets out to critically examine existing evidence to: i) ascertain whether vocalization frequency (volubility) posits a plausible marker of cochlear implantation success in infancy, and ii) determine the clinical usefulness of post-implementation vocalization frequency data in predicting later language development. Only recent peer-reviewed articles with substantial impact on vocalization growth during the first year of life, examining sound production characteristics of normally hearing (NH) and hearing impaired infants fitted with cochlear implantation (CI) were mentioned. Recorded differences in linguistic performance among NH and CI infants are typically attributed to auditory deprivation. Infants who have undergone late CI, produce fewer syllables (low volubility) and exhibit late-onset babbling, especially those who received their CIs at the age of 12 months or thereafter. Contrarily, early recipients (before the 12-month of age) exhibit higher volubility (more vocalizations), triggered from CI-initiated auditory feedback. In other words, early CI provides infants with early auditory access to speech sounds, leading to advanced forms of babbling and increased post-implementation vocalization frequency. Current findings suggest vocalization frequency as a plausible criterion of the success of early CI. It is argued that vocalization frequency predicts language development and affects habilitation therapy.

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Hearing Loss in Children: Clinical-Epidemiological Data from Two Different Provinces of the Same Region

Audiology Research ◽

10.3390/audiolres11020017 ◽

2021 ◽

Vol 11 (2) ◽

pp. 192-199

Author(s):

Silvia Palma ◽

Andrea Ciorba ◽

Laura Nascimbeni ◽

Mariachiara Pecovela ◽

Laura Negossi ◽

...

Keyword(s):

Risk Factors ◽

Hearing Loss ◽

Health Service ◽

Late Onset ◽

Epidemiological Data ◽

Hearing Screening ◽

First Year ◽

Screening Programs ◽

Rehabilitation Programs ◽

First Year Of Life

Background: In many countries, neonatal hearing screening programs (NHS) have been available for many years; however, because of the presence of hearing loss at late onset, early hearing detection programs (EHDP) have been implemented. The aim of this study was to evaluate all cases of infantile hearing loss under the care of two different provinces of a regional health service since the introduction of NHS. Methods: Clinical data (the presence of audiological risk factors, age at which children are placed under the care of health service, entity of hearing loss, treatment, and exposure to bilingualism) were retrospectively collected during the period from 1 January 2012 to 31 December 2018, starting from the IT management system used in all of the regional neuropsychiatric services. Results: In total, 124 children were included—116 cases failed the screening, 1 case had an untraceable result, and 7 cases (5.6%) had hearing screening that passed. Most of the children were placed under the care of a neuropsychiatric infantile and adolescence (NPIA) service within the first year of life. The main differences across the two provinces concerned the percentages of audiological risk factors and the number of unilateral hearing loss cases. Conclusion: In order to plan and manage hearing rehabilitation programs for children in the best way, it is very important to know the local clinical-epidemiological features of the population.

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