Aquaporin 4

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PDB 2d57 EBI.png
Available structures
PDB Ortholog search: PDBe RCSB
Aliases AQP4, aquaporin 4, HMIWC2, MIWC, WCH4
External IDs OMIM: 600308 MGI: 107387 HomoloGene: 37507 GeneCards: 361
Species Human Mouse
RefSeq (mRNA)


RefSeq (protein)


Location (UCSC) Chr 18: 26.85 – 26.87 Mb Chr 18: 15.39 – 15.4 Mb
PubMed search [1] [2]
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Aquaporin 4 also known as AQP4 is a protein which in humans is encoded by the AQP4 gene.[3] AQP4 belongs to the aquaporin family of integral membrane proteins that conduct water through the cell membrane.


AQP4 is constitutively expressed in the basolateral cell membrane of principal collecting duct cells in the kidney and provide a pathway for water to exit these cells.[4]

AQP4 is also expressed in astrocytes and is upregulated by direct insult to the central nervous system.[5]


Aquaporin-4 is the primary autoimmune target in neuromyelitis optica,[6][7] in which AQP4-specific IgG antibodies target astrocytic AQP4, resulting in complement activation and inflammation.[8]

See also[edit]


  1. ^ "Human PubMed Reference:". 
  2. ^ "Mouse PubMed Reference:". 
  3. ^ Jung JS, Bhat RV, Preston GM, Guggino WB, Baraban JM, Agre P (December 1994). "Molecular characterization of an aquaporin cDNA from brain: candidate osmoreceptor and regulator of water balance". Proceedings of the National Academy of Sciences of the United States of America. 91 (26): 13052–6. doi:10.1073/pnas.91.26.13052. PMC 45579free to read. PMID 7528931. 
  4. ^ Agre P, Nielsen S (1996). "The aquaporin family of water channels in kidney". Nephrologie. 17 (7): 409–15. PMID 8987045. 
  5. ^ Nagelhus EA, Mathiisen TM, Ottersen OP (2004). "Aquaporin-4 in the central nervous system: cellular and subcellular distribution and coexpression with KIR4.1". Neuroscience. 129 (4): 905–13. doi:10.1016/j.neuroscience.2004.08.053. PMID 15561407. 
  6. ^ Lennon VA, Kryzer TJ, Pittock SJ, Verkman AS, Hinson SR (August 2005). "IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel". J. Exp. Med. 202 (4): 473–7. doi:10.1084/jem.20050304. PMC 2212860free to read. PMID 16087714. 
  7. ^ Bradl M, Lassmann DH (September 2008). "Anti-aquaporin-4 antibodies in neuromyelitis optica: how to prove their pathogenetic relevance?" (PDF). International MS Journal / MS Forum. 15 (3): 75–8. PMID 18812056. 
  8. ^ Papadopoulos MC, Bennett JL, Verkman AS (Aug 12, 2014). "Treatment of neuromyelitis optica: state-of-the-art and emerging therapies.". Nature reviews. Neurology. 10: 493–506. doi:10.1038/nrneurol.2014.141. PMID 25112508. 

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