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Related Concept Videos

Type IV Collagen of Basal Lamina01:05

Type IV Collagen of Basal Lamina

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Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen  forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
A type IV collagen molecule has six alpha chains which can...
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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
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Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
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Translation01:31

Translation

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Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
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Translation

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Lesson: Translation
Translation is the process of synthesizing proteins from the genetic information carried by messenger RNA (mRNA). Following transcription, it constitutes the final step in the expression of genes. This process is carried out by ribosomes, complexes of protein and specialized RNA molecules. Ribosomes, transfer RNA (tRNA), and other proteins produce a chain of amino acids—the polypeptide—as the end product of translation.
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Mismatch Repair

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Organisms are capable of detecting and fixing nucleotide mismatches that occur during DNA replication. This sophisticated process requires identifying the new strand and replacing the erroneous bases with correct nucleotides. Mismatch repair is coordinated by many proteins in both prokaryotes and eukaryotes.
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Related Experiment Video

Updated: Jan 15, 2026

Mouse Model of Metabolic Dysfunction-Associated Steatotic Liver Disease with Fibrosis
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A collagen defect in homocystinuria.

A H Kang, R L Trelstad

    The Journal of Clinical Investigation
    |October 1, 1973
    PubMed
    Summary

    Homocysteine impairs collagen cross-linking, a key process for connective tissue strength. This study reveals homocysteine

    Area of Science:

    • Biochemistry
    • Connective Tissue Biology
    • Biomolecular Chemistry

    Background:

    • Homocystinuria is characterized by connective tissue abnormalities.
    • Elevated plasma amino acids, including homocysteine, are hallmarks of homocystinuria.
    • The precise biochemical mechanism underlying these connective tissue defects remains incompletely understood.

    Purpose of the Study:

    • To investigate the biochemical mechanism of connective tissue abnormalities in homocystinuria.
    • To examine the effects of accumulated amino acids, particularly homocysteine, on collagen cross-link formation.
    • To elucidate how homocysteine impacts the structural integrity of collagen.

    Main Methods:

    • In vitro studies using purified rat skin collagen incubated with homocysteine, homocystine, or methionine.

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  • Assessment of collagen polymerization and intermolecular cross-link formation via solubility tests and biochemical analysis.
  • Analysis of collagen cross-links and dermal collagen solubility in skin biopsy samples from homocystinuria patients.

    • Main Results:

    • Homocysteine significantly inhibited the formation of insoluble collagen fibrils and reduced key reducible cross-links.
    • The content of precursor aldehydes (allysine) was markedly diminished in collagen incubated with homocysteine.
    • In vitro findings were supported by decreased collagen cross-links and increased collagen solubility in homocystinuria patient samples.

    Conclusions:

    • Homocysteine directly interferes with collagen intermolecular cross-link formation by binding to aldehydic functional groups.
    • This interference disrupts the collagen macromolecular network, leading to connective tissue defects.
    • The study suggests a potential pathogenetic mechanism for connective tissue abnormalities in homocystinuria involving homocysteine-mediated collagen cross-linking defects.