Functions and Diseases of Storage Disorders

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lysosomes is a sac-shaped cell organelle surrounded by a single membrane. Lysosomes contain hydrolytic enzymes that are used by animal cells to break down food into macromolecules.

Citing the book Cell Biology by Rahmadina and Husnarika, this cell organelle was discovered in 1950 by Christian de Duve. Lysosomes are present in eukaryotic cells, with a slightly spherical shape 1.5 m in diameter and bounded by a single membrane. However, sometimes very small lysosomes are also found.

Rough ER creates hydrolytic enzymes and lysosomal membranes which are then transferred to the Golgi apparatus for reprocessing. Golgi bodies will produce lysosomes which contain a lot of protein in them, as described in the Cell Biology Self-Learning Module.

Lysosomal Function

lysosomes has several functions that can be categorized into 3 groups, namely:

  1. Digest food substances. In this category, lysosomes work to destroy foreign food substances that enter / are in the cytoplasm.
  2. Autophagy, namely the breakdown or degradation of cell parts that are no longer functioning.
  3. Autolysis, which destroys the cell itself.

Some of the functions of lysosomes, including:

  1. Lysosomes function to self-destruct by releasing all the enzymes present in lysosomes.
  2. Digest foreign food substances resulting from phagocytosis (solid form) and pinocytosis (liquid form)
  3. Digest spare food
  4. Destroys old or damaged cell organelles so that they can no longer work
  5. Destroys objects that are outside the cell
  6. Destroy foreign substances

Disorders of Lysosomes

Disorders that occur in lysosomes are commonly referred to as lysosomal storage disorders or lysosomal storage disorders (LSD). LSD is a genetic disease that can reduce one or more hydrolase enzymes in lysosomes.

LSD can interfere with the function of enzymes in lysosomes which will result in the accumulation of indigestible particles. LSD is caused by:

Lack of activator enzymes.

Lack of activator protein substrate.

Lack of transport proteins to transport digestive products from lysosomes.

There is an abnormality in the process of post-translational protein enzymes.

LSD is categorized based on the type of substrate that has accumulated. Here are the groupings:

  • Impaired metabolism of glycosaminoglycans (Mucopolysaccharidosis)
  • Damage to glycan degradation of glycoprotein
  • Damage to glycogen degradation
  • Damage to the degradation of sphingolipid components
  • Damage to polypeptide degradation
  • Impaired transport degradation of cholesterol, cholesterol esters, or other lipid complexes
  • Multiple lysosomal enzyme deficiency
  • Damage to transport and exchange

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