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Sickle Cell disease

inherited hemoglobin disorders involving hemoglobin S

A group of inherited blood disorders in which hemoglobin S is present with another disease-associated HBB allele. Low-oxygen HbS can form fibers, making red blood cells rigid and contributing to anemia and blocked blood flow.

MEDLINEPLUS GENETICS IDENTIFIERSickle cell diseaseView source record ↗

Overview

EXPLORE BY LEVEL

affects red blood cells, which carry oxygen. A change in the HBB instructions can make stick together when oxygen is low. The cells can then become stiff and curved, break down early, or slow blood flow through tiny vessels. has several forms, depending on which gene versions a person inherits.

Quick Facts

AT A GLANCE
Main geneHBB
InheritanceUsually autosomal recessive
Affected cellsRed blood cells
TypesHbSS, HbSC, HbS/beta-thalassemia, others

Follow the Connections

See how Sickle Cell disease links to other biological records.

Open network
GeneHBBpathogenic variants causeMolecular complexAdult Hemoglobin AaffectsProcessMendelian Inheritanceillustrated byMolecular complexHemoglobin Scan contribute to
All 6 Structured Connections
HBB · GenePATHOGENIC VARIANTS CAUSE →Sickle Cell disease · Disease
Sickle Cell disease · DiseaseAFFECTS →Adult Hemoglobin A · Molecular complex
Mendelian Inheritance · ProcessILLUSTRATED BY →Sickle Cell disease · Disease
Hemoglobin S · Molecular complexCAN CONTRIBUTE TO →Sickle Cell disease · Disease
Sickle Cell disease · DiseaseASSOCIATED WITH →HBB · Gene
Sickle Cell disease · DiseaseCAN MODIFY MALARIA OUTCOME IN SOME HETEROZYGOTES →Plasmodium falciparum · Taxon

Recommended Reading

Trusted references for learning more about Sickle Cell disease.

databaseSickle cell diseaseMedlinePlus Genetics databaseHBB geneMedlinePlus Genetics databaseAbout sickle cell diseaseCenters for Disease Control and Prevention guideSickle Cell Disease StorylineHHMI BioInteractive
Show 1 more sources
Determination of deoxyhemoglobin S polymer in sickle erythrocytes upon deoxygenationNoble et al. · PMID 6933568 · article
Research Claims

Variants in HBB produce hemoglobin S; sickle cell disease includes HbSS, HbSC, and HbS/beta-thalassemia forms.

Rigid sickle-shaped red cells can break down early and obstruct small vessels, causing pain and other complications.