CD241 RhAG (Rhesus blood group-associated glycoprotein), Rh50,
Molecule TypeAntigen ExpressionMolecular Weight
Min / Max
Lineage Restricted Molecule
Type 3 glycoprotein, 12 span
Erythrocyte
Red Cell
50 / 50

Expression
CD241 is expressed on red blood cells and erythrocytes.

Structure
MOLECULAR FAMILY NAME: Belongs to the rhesus blood group family.

CD241 is a multi-pass type-3, span 12, 409 aa glycoprotein.  It contains an extracellular domain which contains a single N-glycosylation site at Asn37, 12 transmembrane helical domains and a C-terminal cytoplasmic domain which interacts with the erythrocyte cytoskeleton.  CD241 is not palmitoylated like CD240CE and CD240D but is phosphorylated.  CD241 is an ammonium transport protein when it is expressed in yeast. 

MOLECULAR MASS
Cell Type Unreduced Reduced
50 kDa

POST-TRANSCRIPTIONAL MODIFICATION: No information.

POST-TRANSLATIONAL MODIFICATION

CD241 is not palmitoylated like CD240CE and CD240D but is phosphorylated.  CD241 is N-glycosylated and does not carry the blood group antigens.


Ligands
LIGANDS AND MOLECULES ASSOCIATED WITH CD241: No information.

Function
The Rh antigens appear to exist as a complex with CD47, LW and glycophorin B and play a critical role in the CD241 glycoprotein.  Formation of a complex with CD241 is essential for expression of Rh blood group antigens.  The Rh blood group antigens are associated with human erythrocyte membrane proteins, the so-called CD240D polypeptides.  CD241 glycoprotein are coprecipitated with the CD240D polypeptides on immunoprecipitation with anti-Rh-specific mono- and polyclonal antibodies.  The core of the Rh complex is thought to consist of two Rh subunits and two CD241 subunits, to which CD47, CD235b (glycophorin B) and CD242 (LW, ICAM-4) associate noncovalently.  CD241 and Rh antigens C-terminal cytoplasmic domains bind ankyrin-R, thereby linking the complex with the actin-spectrin-based red cell skeleton.  Recently it has been shown that CD241 functions as an ammonium transporter, facilitating the movement of CH3NH2 and NH3 across the erythrocyte membrane.  It may promote erythrocyte mediated retention of ammonium from the plasma and its release to detoxifying organs such as the liver and brain.  It may be part of an oligomeric complex which is likely to have a transport or channel function in the erythrocyte membrane.

BIOCHEMICAL ACTIVITY: No information.

DISEASE RELEVANCE AND FUNCTION OF CD241 IN INTACT ANIMAL

The very rare Rh-null phenotype is the result of inactivation of the RHAG gene.  The absence of CD241 prevents expression of all Rh antigens.  Rh-null individuals have chronic hemolytic anemia of varying severity, an increased osmotic fragility, abnormal cation transport and cell morphology (stomato-spherocytosis), an altered ion transport system and abnormal membrane phospholipid organization. 

Comments
MOLECULAR INTERACTIONS -
PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD241: No information.

SUBSTRATES: No information.

ENZYMES WHICH MODIFY CD241: No information.


Database accession numbers
AnimalPIRSWISSPROTEMGBL/GENBANK
 
HumanEntrezgene 6005Q02094
Antibodies

Revised June 25, 2008


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