Anti-MFAP1 Antibody Picoband® Fluoro647 Conjugated

Microfibrillar-associated protein 1 is a protein that in humans is encoded by the MFAP1 gene. Microfibrils are an important component of the extracellular matrix of many tissues and can either associate with or without elastin. Several microfibril associated proteins (MFAPs) have been cloned, including MFAP1, MFAP3 and MFAP4. The MFAP1 and MFAP3 genes are localized near the fibrillin genes FBN1 and FBN2, respectively. Mutations in FBN1 are linked to Marfan syndrome. Mutations in FBN2 have been linked to congenital contractural arachnodactyly. This suggests roles for MFAP1 and MFAP3 in heritable diseases affecting microfibrils. Deletion of MFAP4 was found in 30 of 31 patients with Smith-Magenis syndrome (SMS), a clinically recognizable multiple congenital anomaly/mental retardation syndrome

Expressed in immune system and fetal brain, but not in other tissues tested or in multiple hematopoietic cell lines. Predominantly expressed in skin keratinocytes but not in fibroblasts, endothelial cells or melanocytes. Increased in lesional psoriasis skin.

Cytokine that binds to and signals through the IL1RL2/IL-36R receptor which in turn activates NF-kappa-B and MAPK signaling pathways in target cells linked to a pro-inflammatory response. Part of the IL-36 signaling system that is thought to be present in epithelial barriers and to take part in local inflammatory response; similar to the IL-1 system with which it shares the coreceptor IL1RAP. Seems to be involved in skin inflammatory response by acting on keratinocytes, dendritic cells and inly on T-cells to drive tissue infiltration, cell maturation and cell proliferation. In cultured keratinocytes induces the expression of macrophage, T-cell, and neutrophil chemokines, such as CCL3, CCL4, CCL5, CCL2, CCL17, CCL22, CL20, CCL5, CCL2, CCL17, CCL22, CXCL8, CCL20 and CXCL1, and the production of proinflammatory cytokines such as TNF-alpha, IL-8 and IL-6. In cultured monocytes upregulates expression of IL-1A, IL-1B and IL-6. In myeloid dendritic cells involved in cell maturation by upregulating surface expression of CD83, CD86 and HLA-DR. In monocyte-derived dendritic cells facilitates dendritic cell maturation and drives T-cell proliferation. May play a role in proinflammatory effects in the lung.

1. Horrigan, S. K., Rich, C. B., Streeten, B. W., Li, Z.-Y., Foster, J. A. Characterization of an associated microfibril protein through recombinant DNA techniques. J. Biol. Chem. 267: 10087-10095, 1992. 2. Kittler, R., Putz, G., Pelletier, L., Poser, I., Heninger, A.-K., Drechsel, D., Fischer, S., Konstantinova, I., Habermann, B., Grabner, H., Yaspo, M.-L., Himmelbauer, H., Korn, B., Neugebauer, K., Pisabarro, M. T., Buchholz, F. An endoribonuclease-prepared siRNA screen in human cells identifies genes essential for cell division. Nature 432: 1036-1040, 2004. 3. Liu, W., Faraco, J., Qian, C., Francke, U. The gene for microfibril-associated protein-1 (MFAP1) is located several megabases centromeric to FBN1 and is not mutated in Marfan syndrome. Hum. Genet. 99: 578-584, 1997.