Part 4: DNA Repair Mechanisms

Maintaining Genome Integrity

DNA is constantly damaged by environmental factors and metabolic byproducts. Cells have evolved sophisticated repair mechanisms to maintain genomic integrity. Defects in DNA repair are associated with cancer, aging, and genetic disorders.

Types of DNA Damage

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Oxidative Damage

Cause: ROS, metabolic byproducts

Example: 8-oxoguanine

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Alkylation

Cause: Alkylating agents

Example: O6-methylguanine

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UV Damage

Cause: Ultraviolet radiation

Example: Thymine dimers

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Deamination

Cause: Spontaneous hydrolysis

Example: C โ†’ U conversion

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Double-strand breaks

Cause: Ionizing radiation, replication errors

Example: Chromosome breaks

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Mismatches

Cause: Replication errors

Example: G-T mispairing

Repair Pathways

Base Excision Repair (BER)

Repairs small, non-helix-distorting lesions

DNA glycosylase โ†’ AP endonuclease โ†’ Pol ฮฒ โ†’ Ligase

Nucleotide Excision Repair (NER)

Repairs bulky, helix-distorting lesions (UV damage)

Recognition โ†’ Incision โ†’ Excision (~30 nt) โ†’ Resynthesis โ†’ Ligation

Mismatch Repair (MMR)

Corrects replication errors and small insertions/deletions

MutS (recognition) โ†’ MutL โ†’ MutH (strand discrimination) โ†’ Excision โ†’ Resynthesis

Double-Strand Break Repair

NHEJ

Non-homologous end joining. Fast but error-prone.

HR

Homologous recombination. Accurate but requires template.

Clinical Significance

Xeroderma Pigmentosum

Defective NER โ†’ UV sensitivity, skin cancer

BRCA1/2 Mutations

Defective HR โ†’ breast/ovarian cancer

Lynch Syndrome

Defective MMR โ†’ colorectal cancer

Ataxia Telangiectasia

Defective ATM โ†’ radiation sensitivity