Cellular structure and function

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Cellular structure and function

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A group of researchers is studying the pathophysiology of Zellweger syndrome, an autosomal recessive disorder of peroxisome biogenesis due to mutated PEX genes. Which of the following best describes the normal function of peroxisomes?  

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The cell is the basic unit that makes up every living organism.

It’s the smallest form of life that can replicate on its own, but cells in our body differ quite a lot from one another.

The human body alone has over 200 distinct cell types - from long skinny neurons that can get over 1 meter long to macrophages that gobble up pathogens to myocytes that contract to let you to flex your muscles.

But despite their differences, they share lots of similar features.

So let’s imagine the average cell as a small apartment. First, we want some walls to distinction between what’s “outside” and what’s “inside.” These walls are the cell membrane or plasma membrane, and they’re made out of a double layer of phospholipid molecules.

Phospholipids have a “head” made out of negatively charged phosphate, which is hydrophilic - meaning it likes water.

Phospholipids also have a “tail” made out of two fatty acids, which are hydrophobic - meaning, they avoid water.

In water, phospholipids form a bilayer - where the hydrophobic tails are oriented inwards, where there is no water, and the hydrophilic heads oriented outwards, in contact with water molecules.

So the plasma membrane forms a wall with water on both sides.

This wall is semipermeable - meaning it allows some things through, like oxygen or carbon dioxide - but doesn’t allow other things through, like glucose and sugars.

Fortunately, we have the “doors” and “windows” on this apartment, and they’re made of special protein channels that are, essentially, tiny tunnels through the phospholipid bilayer.

These channels allow water and specific ions like sodium and potassium to come in and out of the cell.

Now, like any well built apartment, our cell has a sturdy framework called the cytoskeleton.

The cytoskeleton is made out of proteins like microfilaments, microtubules, and intermediate filaments, which all provide structural stability.

The cytoskeleton is also very dynamic, allowing the cell to change shape by selectively contracting and extending filaments - which is important in some cell functions, like muscle contraction, cell division, and even cell movement!

The cytoskeleton also helps structures within the cell move from one area to another.

Now that the apartment is built - let’s fill it in. The cell is filled with intracellular fluid called cytosol, which contains various ions like sodium and potassium.

In addition, cells have a number of organelles, which are like little rooms within our apartment.

And together, the cytosol and the organelles make up the cytoplasm of the cell.

Now, the nucleus is a highly specialized organelle, which is home to our genetic material.

In terms of rooms, you can picture the nucleus as the office, with bookshelves containing various books and manuals. How to do home repair, how to do interior remodeling, how to talk to your neighbors, how to take out the trash, and so on.

Extending the analogy, each book would be like a gene that contains the exact information - like a blueprint - needed to build a specific protein.

Drilling down deeper - the exact letters and words of the books are written in the language of DNA.

Now, as it turns out DNA is actually one long molecule with some parts that code for genes connected together by other parts that don’t code for genes.

If the DNA molecule in a cell were stretched out, it would be about 2 meters long!

Fortunately, the DNA is tightly coiled and wound in multiple steps so that it packs down really tight.

First off, it’s coiled around proteins called histones.

Zoomed in to that level, the DNA looks like pearls on a string. But then that “necklace” gets folded further and further, creating an extremely neatly folded and tightly packed form of DNA, called a chromosome.

Now, to make a protein, the DNA of a particular gene has to be transcribed, or copied over into RNA - specifically messenger RNA or mRNA.

Special structures inside the cell, called ribosomes, can then use the mRNA copies to produce proteins!

The ribosomes are made by a structure within the nucleus called the nucleolus out of ribosomal RNA or rRNA and proteins.

Summary

Cells are the smallest structural and functional unit of life. All living things are made up of cells, from single-celled organisms to the largest animals. Cells have small parts called organelles that carry out vital functions necessary for a cell's life. Major organelles include: The cell membrane: surrounds and protects the cell. The nucleus: contains the DNA. The endoplasmic reticulum: helps in protein synthesis, lipids, and steroids. The mitochondria: are responsible for providing the cell with energy.