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Biochemistry & Molecular Biology
Francesco Chiuso, Rossella delle Donne, Giuliana Giamundo, Laura Rinaldi, Domenica Borzacchiello, Federica Moraca, Daniela Intartaglia, Rosa Iannucci, Emanuela Senatore, Luca Lignitto, Corrado Garbi, Paolo Conflitti, Bruno Catalanotti, Ivan Conte, Antonio Feliciello
Summary: Bardet-Biedl syndrome (BBS) is a ciliopathy characterized by retinal degeneration, obesity, renal abnormalities, postaxial polydactyly, and developmental defects. The E3 ubiquitin ligase PJA2 has been identified as a regulator of the BBSome, an octameric complex that controls ciliary trafficking. Ubiquitylation of BBS1 by PJA2 stabilizes the BBSome and promotes its binding to BBS3, leading to proper ciliary membrane targeting. Disruption of PJA2 or expression of a ubiquitylation-defective BBS1 mutant affects GPCR trafficking and gene transcription, recapitulating the BBS phenotype in a medaka fish model.
Article
Biochemistry & Molecular Biology
Michael P. Duffy, McKenzie E. Sup, X. Edward Guo
Summary: The study demonstrates the role of AC3 in osteocyte mechanotransduction, particularly through a signaling axis involving primary cilia and GSK3 beta. Inhibiting AC3 leads to decreased cAMP signaling and enhanced osteogenic response to longer durations of fluid flow stimulation. Further investigation is warranted to explore AC3 as a potential therapeutic target for bone diseases like osteoporosis.
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
(2021)
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Physiology
Steven J. Kleene
Summary: Polycystic kidney disease (PKD) is a leading cause of end-stage renal disease. Researchers have discovered that regenerative signaling of intraciliary Ca2+ may reduce the development of PKD.
FRONTIERS IN PHYSIOLOGY
(2022)
Article
Biology
Janani Gopalan, Mitchell H. Omar, Ankita Roy, Nelly M. Cruz, Jerome Falcone, Kiana N. Jones, Katherine A. Forbush, Jonathan Himmelfarb, Benjamin S. Freedman, John D. Scott
Summary: This study demonstrates that the AKAP220-PPI-HDAC6 pathway plays a critical role in primary cilia development, and renal failure may be associated with pathophysiological defects in water homeostasis and genetic disorders.
Article
Biochemistry & Molecular Biology
Fatmanur Tiryaki, Jovana Deretic, Elif Nur Firat-Karalar
Summary: Centrioles and cilia are essential microtubule-based structures in cells that play critical roles in cell function and development. This study identifies a new microtubule-associated protein, ENKD1, which regulates microtubule organization and stability. Furthermore, it shows that ENKD1 depletion leads to defective regulation of ciliary length and content, as well as a failure to respond to Hedgehog pathway activation.
Article
Physiology
Steven J. Kleene, Nancy K. Kleene
Summary: Research suggests that 15% of cases of autosomal dominant polycystic kidney disease result from defects in PC2. Recent experiments show that native PC2 channels in renal primary cilia can allow a small influx of Ca2+, which may have implications for disease progression.
AMERICAN JOURNAL OF PHYSIOLOGY-RENAL PHYSIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Masaki Saito, Marina Hirano, Tomohiro Izumi, Yu Mori, Kentaro Ito, Yurika Saitoh, Nobuo Terada, Takeya Sato, Jun Sukegawa
Summary: This study identified the specific expression of 4.1G in bone and demonstrated its essential role in osteoblast differentiation. 4.1G promotes osteoblast differentiation by organizing primary cilia and participating in cilia-mediated Hedgehog signaling.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Physiology
Ester Paolocci, Manuela Zaccolo
Summary: cAMP is a universal second messenger that controls cellular functions via precise regulation. Dysfunction of ciliary signaling leads to ciliopathies like ADPKD. Recent studies suggest that cAMP generated in the primary cilium drives cyst formation in ADPKD, indicating the cilium is an independent compartment for cAMP signaling. The localization of GPCRs in the ciliary membrane determines their downstream function, and optogenetic tools have contributed to establish the role of cAMP in cystogenesis.
FRONTIERS IN PHYSIOLOGY
(2023)
Article
Biology
Cristina Salmeron, Till S. Harter, Garfield T. Kwan, Jinae N. Roa, Salvatore D. Blair, Jodie L. Rummer, Holly A. Shiels, Greg G. Goss, Rod W. Wilson, Martin Tresguerres
Summary: The study identified sAC genes in various fish species and focused on rainbow trout sAC, finding multiple potential protein isoforms. Biochemical analyses showed that rtsAC protein is sensitive to HCO3- stimulation, with different responses to inhibitors and modulators, indicating its potential important role in fish.
Editorial Material
Cell Biology
Danielle T. Arena, Aldebaran M. Hofer
Summary: The primary cilium has the ability to generate and interpret cAMP signals separately from the rest of the cell body, affecting transcriptional activity via the hedgehog pathway and influencing zebrafish development. Computer modeling explains how the geometry of the cilium allows it to filter out cAMP signals from the cell body to receive messages specific to the cilium.
Article
Dermatology
Gabriela Blanchard, Christine Pich, Daniel Hohl
Summary: Primary cilium (PC) is a microtubule-based organelle that plays an important role in development and tissue homeostasis. Ciliopathies are a group of human diseases characterized by disordered cilium. In this study, it was found that HaCaT cells, a model for studying epidermal homeostasis, can efficiently develop cilia. The detailed progression of ciliation in HaCaT cells can serve as a basis for further ciliary studies in this cell line.
EXPERIMENTAL DERMATOLOGY
(2022)
Article
Microbiology
Jakub Zahumensky, Caroline Mota Fernandes, Petra Vesela, Maurizio Del Poeta, James B. Konopka, Jan Malinsky
Summary: The protein Nce102 has been identified as a marker of local sphingolipid levels in both Saccharomyces cerevisiae and Candida albicans, indicating a conserved principle of local sphingolipid control across species and potential applications for novel therapeutic approaches.
MICROBIOLOGY SPECTRUM
(2022)
Review
Biochemistry & Molecular Biology
Sudheendra Hebbar Subramanyam, Klaus Tenbrock
Summary: CREM is a transcriptional regulator in CD4(+) T cells, influencing the expression of various effector cytokines and playing a crucial role in T cell functions, particularly in autoimmune diseases.
BIOLOGICAL CHEMISTRY
(2021)
Article
Genetics & Heredity
Wolfgang H. Ziegler, Sarah Luediger, Fatima Hassan, Margarita E. Georgiadis, Kathrin Swolana, Amrit Khera, Arne Mertens, Doris Franke, Kai Wohlgemuth, Mareike Dahmer-Heath, Jens Koenig, Claudia Dafinger, Max C. Liebau, Metin Cetiner, Carsten Bergmann, Birga Soetje, Dieter Haffner
Summary: In pediatric hereditary cystic kidney diseases, the study of urine-derived renal tubular epithelial cells (URECs) can help identify gene and variant-specific properties, and determine aspects of epithelial dysfunction. URECs from patients show characteristics and behavior related to the individual genotype, while no predictable and satisfactory outcome could be established in healthy controls.
ORPHANET JOURNAL OF RARE DISEASES
(2022)
Article
Biology
Petra Zur Lage, Zhiyan Xi, Jennifer Lennon, Iain Hunter, Wai Kit Chan, Alfonso Bolado Carrancio, Alex von Kriegsheim, Andrew P. Jarman
Summary: Ciliary motility in humans relies on highly conserved axoneme-specific dynein motor complexes, and mutations in these motors can lead to primary ciliary dyskinesia (PCD). Research in Drosophila has identified a less-known assembly factor, DNAAF3, which is essential for the function of mechanosensory neurons and sperm-producing cells in fruit flies. Mutations in DNAAF3 result in deaf larvae and uncoordinated adults, indicating a dysfunction in Ch neurons. Additionally, the absence of dynein arms in mutant Ch neuron cilia and immotile sperm with disrupted axoneme further demonstrates the importance of dynein motors in ciliary function and sperm motility.
Review
Urology & Nephrology
Surya M. Nauli, Rajasekharreddy Pala, Steven J. Kleene
CURRENT OPINION IN NEPHROLOGY AND HYPERTENSION
(2016)
Review
Biochemistry & Molecular Biology
Rajasekharreddy Pala, Nedaa Alomari, Surya M. Nauli
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2017)
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Materials Science, Biomaterials
Pala Rajasekharreddy, Pathipati Usha Rani, Saidulu Mattapally, Sanjay Kumar Banerjee
MATERIALS SCIENCE AND ENGINEERING C-MATERIALS FOR BIOLOGICAL APPLICATIONS
(2017)
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Immunology
Jobina Rajkumari, Siddhardha Busi, Aneesh Cherukunnel Vasu, Palarajasekhar Reddy
MICROBIAL PATHOGENESIS
(2017)
Review
Radiology, Nuclear Medicine & Medical Imaging
Yun Zeng, Jing Zhu, Junqing Wang, Paramanantham Parasuraman, Siddhardha Busi, Surya M. Nauli, Yi Xiang J. Wang, Rajasekharreddy Pala, Gang Liu
QUANTITATIVE IMAGING IN MEDICINE AND SURGERY
(2018)
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Chemistry, Multidisciplinary
Rajasekharreddy Pala, Ashraf M. Mohieldin, Kiumars Shamloo, Rinzhin T. Sherpa, Sarmed H. Kathem, Jing Zhou, Zhongyue Luan, Jian-Guo Zheng, Amir Ahsan, Surya M. Nauli
Review
Cell Biology
Rajasekharreddy Pala, Maha Jamal, Qamar Alshammari, Surya M. Nauli
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Chemistry, Multidisciplinary
Rajasekharreddy Pala, Ashraf M. Mohieldin, Rinzhin T. Sherpa, Sarmed H. Kathem, Kiumars Shamloo, Zhongyue Luan, Jing Zhou, Jian-Guo Zheng, Amir Ahsan, Surya M. Nauli
Review
Biochemistry & Molecular Biology
Pala Rajasekharreddy, Chao Huang, Siddhardha Busi, Jobina Rajkumari, Ming-Hong Tai, Gang Liu
CURRENT MEDICINAL CHEMISTRY
(2019)
Article
Microbiology
Busi Siddhardha, Uday Pandey, K. Kaviyarasu, Rajasekharreddy Pala, Asad Syed, Ali H. Bahkali, Abdallah M. Elgorban
Review
Nanoscience & Nanotechnology
Rajasekharreddy Pala, V. T. Anju, Madhu Dyavaiah, Siddhardha Busi, Surya M. Nauli
INTERNATIONAL JOURNAL OF NANOMEDICINE
(2020)
Review
Pharmacology & Pharmacy
Rajasekharreddy Pala, Subhaswaraj Pattnaik, Siddhardha Busi, Surya M. Nauli
Summary: Cardiovascular diseases are the leading cause of death globally, emphasizing the importance of early diagnosis and treatment. The advent of nanotechnology has revolutionized disease understanding and treatment, with the concept of theranostics gaining attention in managing CVDs.
Article
Biochemistry & Molecular Biology
Young -Woo Nam, Rajasekharreddy Pala, Naglaa Salem El-Sayed, Denisse Larin-Henriquez, Farideh Amirrad, Grace Yang, Mohammad Asikur Rahman, Razan Orfali, Myles Downey, Keykavous Parang, Surya M. Nauli, Miao Zhang
Summary: Compound 4 selectively potentiates human K(Ca)2.3 channels, increasing flow-induced Ca2+ signaling and cilia length in endothelial cells, with potential implications in vasodilation and ciliopathic hypertension.
ACS CHEMICAL BIOLOGY
(2022)
Article
Physiology
Kiumars Shamloo, Juan Chen, Jasmine Sardar, Rinzhin T. Sherpa, Rajasekharreddy Pala, Kimberly F. Atkinson, William J. Pearce, Lubo Zhang, Surya M. Nauli
FRONTIERS IN PHYSIOLOGY
(2017)