What does AQP4 do?

What does AQP4 do?

AQP4 plays a critical role in brain and spinal cord water balance, astrocyte migration, neural signal transduction, and neuroinflammation. It is involved in the development, function, and integrity of the interface between the brain and blood and between the brain and cerebrospinal fluid.

Where is AQP4?

Specifically within the central nervous system (CNS), AQP4 can be found along the spinal cord and serves as the main water channel. The AQP4 channels are highly concentrated in the blood-brain barrier (BBB), as well as in other cerebrospinal fluid barriers.

What is NMO AQP4?

Antibodies to aquaporin-4 (called NMO-IgG or AQP4-Ab) constitute a sensitive and highly specific serum marker of neuromyelitis optica (NMO) that can facilitate the differential diagnosis of NMO and classic multiple sclerosis.

How many people in the world have NMO?

There are an estimated 4,000 people with NMO in the United States and a quarter-million people worldwide.

What happens if aquaporin stops working?

When plant aquaporins are silenced, the hydraulic conductance and photosynthesis of the leaf decrease. When gating of plant aquaporins occurs, it stops the flow of water through the pore of the protein.

What causes anti MOG?

About anti-MOG syndrome. Anti-MOG syndrome is an autoimmune disorder in which the immune system mistakenly attacks myelin oligodendrocyte glycoprotein (MOG). This protein is located on the surface of myelin, an insulating layer that protects nerve cells and helps facilitate communication between them.

What is the life expectancy of NMO?

Neuromyelitis optica life expectancy The life expectancy of a person with NMO varies widely. Past studies have suggested that the natural 5-year mortality rate for NMO is about 22–30%, according to a 2021 research review. More recent research suggests that with treatment the rate declines to 3–7%.

Can the NMO be reversed?

Neuromyelitis optica can’t be cured, though long-term remission is sometimes possible with the right management. NMO treatment involves therapies to reverse recent symptoms and prevent future attacks.

What type of protein is aquaporin?

integral membrane proteins
Aquaporins (AQP) are integral membrane proteins that serve as channels in the transfer of water, and in some cases, small solutes across the membrane. They are conserved in bacteria, plants, and animals. Structural analyses of the molecules have revealed the presence of a pore in the center of each aquaporin molecule.

What condition might result from an excess of aquaporins?

What condition might result from an excess of aquaporins? fluid retention in pregnant women ( Aquaporins transport water. An excess of aquaporins during pregnancy can result in fluid retention.) Water movement is important in urine formation in the kidneys.

How do you get NMO disease?

The cause of neuromyelitis optica is usually unknown, although it sometimes appears after an infection, or it can be associated with another autoimmune condition. Neuromyelitis optica is often misdiagnosed as multiple sclerosis (MS) or perceived as a type of MS , but NMO is a distinct condition.

What are AQP4 proteins?

AQP4 proteins are water channels expressed in astrocytes and other cells, and organize into large molecular aggregates called orthogonal arrays of particles ( Jung et al., 1994; Rossi et al., 2012 ).

What is AQP4 antibody-positive NMO?

Antibodies against the astrocyte water channel protein, AQP4, were first described in 2004 in patients with the rare inflammatory demyelinating disease, NMO ( Lennon et al., 2004 ). The clinical features and imaging abnormalities reported in children with AQP4 antibody-positive NMO are similar to the adult phenotype ( McKeon et al., 2008 ).

What is AQP4-IgG?

AQP4-IgG are polyclonal, belong to the IgG1 class, and are able to mediate complement- and cell-mediated cytotoxicity. A role for both mechanisms has been confirmed in animal models of NMOSD and products of complement activation are one of the neuropathological diagnostic hallmarks of the disease.

Do recombinant human anti-AQP4 antibodies cause astrogliopathy with loss of AQP4?

In the setting of experimental autoimmune encephalomyelitis the recombinant human anti-AQP4 antibodies caused astrogliopathy with loss of AQP4, demonstrating for the first time in an experimental system a role for an antigen-specific human autoantibody in the pathogenesis of a CNS demyelinating disease.