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Related Concept Videos

Bone Disorders01:29

Bone Disorders

Aging and its effect on bone remodeling is the most common cause of bone disorders. In young and healthy people, bone deposition and resorption happen at an equal rate to maintain optimal bone health.
Bone deposition is also affected by the levels of sex hormones like estrogen and testosterone that promote osteoblast activity and bone matrix synthesis. When the level of these hormones decreases due to aging, it causes a reduction in bone deposition. As a result, bone resorption by osteoclasts...
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Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
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Bone Remodeling

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What is the Skeletal System?01:02

What is the Skeletal System?

Overview
Osteoclasts in Bone Remodeling01:31

Osteoclasts in Bone Remodeling

Osteoclasts are cells responsible for bone resorption and remodeling. They originate from hematopoietic progenitor cells present in the bone marrow. Numerous progenitor cells fuse to form multinucleated cells, each with 10-20 nuclei. A single osteoclast has a diameter of 150 to 200 µM. These cells have ruffled borders that break down the underlying bone tissue and release minerals such as calcium into the blood in bone resorption. Osteoclasts cling to bones with their ruffled edges during bone...
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Chronic Kidney Disease II: Clinical Manifestations

Chronic Kidney Disease (CKD) progressively impairs multiple body systems due to the accumulation of uremic toxins, which disrupt cellular functions across various organs.Neurologic symptomsNeurologic symptoms often arise early in CKD, as uremic toxin buildup drives changes in cognitive and motor functions. Patients frequently experience fatigue, headache, confusion, difficulty concentrating, and, in severe cases, seizures. Peripheral neuropathy commonly manifests as burning sensations in the...

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Related Experiment Video

Updated: May 7, 2026

Skeletal Phenotype Analysis of a Conditional Stat3 Deletion Mouse Model
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Progressive destructive bone changes in patients with cystinosis.

Maria Klusmann1, William Van't Hoff, Fergal Monsell

  • 1Radiology, Imaging Department, University College Hospital, London, UK.

Skeletal Radiology
|October 1, 2013
PubMed
Summary

Nephropathic cystinosis, a genetic disorder causing cystine buildup, leads to severe Fanconi syndrome and progressive skeletal issues. This study details previously unreported bony abnormalities in affected patients, highlighting the disorder's complex impact.

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Area of Science:

  • Biochemistry
  • Genetics
  • Pediatrics

Background:

  • Nephropathic cystinosis is an autosomal recessive lysosomal storage disorder.
  • Intracellular cystine accumulation results from CTNS gene mutations.
  • Clinical features include Fanconi syndrome, growth retardation, and organ damage.

Purpose of the Study:

  • To describe novel progressive bony abnormalities in nephropathic cystinosis.
  • To enhance understanding of the skeletal manifestations of this rare genetic disorder.

Main Methods:

  • Case study of three unrelated patients with nephropathic cystinosis.
  • Detailed clinical and radiological assessment of skeletal features.

Main Results:

  • Three patients presented with unique progressive bony abnormalities.
  • These findings expand the known skeletal complications of nephropathic cystinosis.

Conclusions:

  • Nephropathic cystinosis can cause previously undocumented skeletal deformities.
  • Further research is needed to fully characterize and manage these bony abnormalities.