A descriptive analysis of 14 cases of progressive-psuedorheumatoid-arthropathy of childhood from south India: Review of literature in comparison with Juvenile Idiopathic Arthritis

Ekbote, Alka V.; Danda, Debashish; Kumar, Sathish; Danda, Sumita; Madhuri, Vrisha; Gibikote, Sridhar

doi:10.1016/j.semarthrit.2012.09.001

Cited by 30 publications

(42 citation statements)

References 13 publications

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“…Given that CCN6 regulates mitochondrial respiration and ATP synthesis, why do specific phenotypes resulting from CCN6 mutations manifest primarily as loss of cartilage during puberty (Dalal et al, 2012;Ekbote et al, 2013;Garcia Segarra et al, 2012;Hurvitz et al, 1999;Liu et al, 2015;Luo et al, 2015;Yang et al, 2013)? Although it is true that the comprehension of CCN6-mutant phenotypes cannot be directly drawn through extrapolation of experimental findings related to wild-type CCN6, a clear knowledge of the function of the wild-type protein nevertheless could be required for a detailed analysis of the mutants.…”

Section: Discussionmentioning

confidence: 99%

“…Mutations within the CCN6 coding sequence are linked to a skeletal disorder termed progressive pseudo rheumatoid dysplasia (PPRD), which is characterized by progressive cartilage loss and irregularities in bone growth (Dalal et al, 2012;Ekbote et al, 2013;Garcia Segarra et al, 2012;Hurvitz et al, 1999;Liu et al, 2015;Luo et al, 2015;Yang et al, 2013). The several mutations associated with PPRD, identified thus far, span across the different domains of CCN6, implying that all the domains of CCN6 contribute to its overall function.…”

Section: Introductionmentioning

confidence: 99%

“…Perhaps the loss of a functional cartilage and the aberrant matrix mineralization associated with CCN6 mutations in individuals with PPRD (Dalal et al, 2012;Ekbote et al, 2013;Garcia Segarra et al, 2012;Hurvitz et al, 1999;Liu et al, 2015;Luo et al, 2015;Yang et al, 2013) are due to an imbalance in cell differentiation and/or hypertrophy, arising partly from altered mitochondrial function. Accordingly, it is essential to estimate the influence of wild-type CCN6 on the activity of mitochondrial electron transport.…”

Section: Introductionmentioning

confidence: 99%

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CCN6 regulates mitochondrial function

Patra

Mahata

Padhan

et al. 2016

Journal of Cell Science

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Despite established links of CCN6, or Wnt induced signaling protein-3 (WISP3), with progressive pseudo rheumatoid dysplasia, functional characterization of CCN6 remains incomplete. In light of the documented negative correlation between accumulation of reactive oxygen species (ROS) and CCN6 expression, we investigated whether CCN6 regulates ROS accumulation through its influence on mitochondrial function. We found that CCN6 localizes to mitochondria, and depletion of CCN6 in the chondrocyte cell line C-28/I2 by using siRNA results in altered mitochondrial electron transport and respiration. Enhanced electron transport chain (ETC) activity of CCN6-depleted cells was reflected by increased mitochondrial ROS levels in association with augmented mitochondrial ATP synthesis, mitochondrial membrane potential and Ca 2+. Additionally, CCN6-depleted cells display ROS-dependent PGC1α (also known as PPARGC1A) induction, which correlates with increased mitochondrial mass and volume density, together with altered mitochondrial morphology. Interestingly, transcription factor Nrf2 (also known as NFE2L2) repressed CCN6 expression. Taken together, our results suggest that CCN6 acts as a molecular brake, which is appropriately balanced by Nrf2, in regulating mitochondrial function.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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CCN6 regulates mitochondrial function

Patra

Mahata

Padhan

et al. 2016

Journal of Cell Science

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“…Painful or swollen joints are characteristic of infl ammatory joint disease, the commonest of which is juvenile idiopathic arthritis; however, juvenile psoriatic arthritis, progressive pseudorheumatoid arthropathy of childhood, and the rare conditions such as carpal-tarsal osteolysis and neonatal-onset multisystem infl ammatory disease (NOMID) may manifest with deformed painful joints (Kim et al 2014 ;Choi et al 1993 ;Ekbote et al 2013 ). Metaphyseal deformities are more likely to be related to growth abnormalities.…”

Section: If the Deformities Are In The Bones Are They Mainly In The mentioning

confidence: 99%

Paediatric Orthopaedic Diagnosis

Joseph

Robb

Loder

et al. 2015

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“…In addition, polymorphisms in WISP-3 have been shown to confer susceptibility to Juvenile Idiopathic Arthritis. 46 …”

Section: Wnt-induced Signaling Protein 3 (Wisp-3)mentioning

confidence: 99%

Wnts’ fashion statement: from body stature to dysplasia

Malhotra

Yang

2014

BoneKEy Reports

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Bone is constantly being made and remodeled to maintain bone volume and calcium homeostasis. Even small changes in the dosage, location and duration of int/Wingless (Wnt) signaling affect skeletal development and homeostasis. Wnt/b-catenin signaling controls cell fate determination, proliferation and survival by affecting a balance between boneforming osteoblast and bone-resorbing osteoclast cell differentiation. During early skeletal development, Wnt/b-catenin signaling is required in directing mesenchymal progenitor cells toward the osteoblast lineage. Later, Wnt/b-catenin in chondrocytes of the growth plate promotes chondrocyte survival, hypertrophic differentiation and endochondral ossification. Gain-or loss-of-function mutations in the Wnt signaling components are causally linked to high or low bone mass in mice and humans. Inactivation of Wnt/b-catenin signaling leads to imbalance between bone formation and resorption because of accelerated osteoclastogenesis due to decline in the levels of osteoprotegerin (OPG) secreted by osteoblasts or directly via Frizzled 8 (Fzd8). In this review, we provide a landscape of the Wnt pathway components in influencing progenitor cell differentiation toward osteoblasts or osteoclasts under physiological conditions as well as pathological disorders resulting in various skeletal dysplasia syndromes.

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A descriptive analysis of 14 cases of progressive-psuedorheumatoid-arthropathy of childhood from south India: Review of literature in comparison with Juvenile Idiopathic Arthritis

Cited by 30 publications

References 13 publications

CCN6 regulates mitochondrial function

CCN6 regulates mitochondrial function

Paediatric Orthopaedic Diagnosis

Wnts’ fashion statement: from body stature to dysplasia

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