Anti-Glial fibrillary acidic protein δ

P14136

FUNCTION: SwissProt: P14136 # GFAP, a class-III intermediate filament, is a cell- specific marker that, during the development of the central nervous system, distinguishes astrocytes from other glial cells.
SIZE: 432 amino acids; 49880 Da
SUBUNIT: Isoform 3 interacts with N-terminus of PSEN1.
SUBCELLULAR LOCATION: Cytoplasm. Note=Associated with intermediate filaments.
DISEASE: SwissProt: P14136 # Defects in GFAP are a cause of Alexander disease [MIM:203450]. Alexander disease is a rare disorder of the central nervous system. It is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of Rosenthal fibers which are cytoplasmic inclusions in astrocytes. The most common form affects infants and young children, and is characterized by progressive failure of central myelination, usually leading to death usually within the first decade. Infants with Alexander disease develop a leukoencephalopathy with macrocephaly, seizures, and psychomotor retardation. Patients with juvenile or adult forms typically experience ataxia, bulbar signs and spasticity, and a more slowly progressive course.
SIMILARITY: SwissProt: P14136 ## Belongs to the intermediate filament family. ... masquer » En voir davantage »

NM_002055.2

This gene encodes one of the major intermediate filament proteins of mature astrocytes. It is used as a marker to distinguish astrocytes from other glial cells during development. Mutations in this gene cause Alexander disease, a rare disorder of astrocytes in the central nervous system. An additional transcript variant has been described, but its full length sequence has not been determined.

Anti-Glial fibrillary acidic protein δ

Human Glial Fibrillary Acidic Protein delta (GFAP-delta) is a GFAP protein isoform that is encoded by an alternative splice variant of the GFAP-gene. As a result, GFAP-delta protein differs from the predominant splice form, GFAP-alpha, by its C-terminal protein sequence. GFAP-delta protein is not expressed by all GFAP expressing astrocytes but specifically by a subpopulation located in the subpial zone of the cerebral cortex, the subgranular zone of the hippocampus and, most intensely, by a ribbon of astrocytes following the ependymal layer of the cerebral ventricles. Therefore, at least in the sub ventricular zone (SVZ), GFAP-delta specifically marks the population of astrocytes that contain the neural stem cells in the adult human brain. Interestingly, the SVZ astrocytes actively splice GFAP-delta transcripts, in contrast to astrocytes adjacent to this layer. Data shows that GFAP-delta protein, unlike GFAP-alpha, is not upregulated in astrogliosis. Data indicates a different functional role for GFAP-delta in astrocyte physiology. Transfection studies showed that GFAP-delta protein expression has a negative effect on GFAP filament formation, and therefore could be important for modulating intermediate filament cytoskeletal properties, possibly facilitating astrocyte motility. Further studies on GFAP-delta and the cells that express it are important for gaining insights into its function during differentiation, migration and during health and disease (Roelofs, RF, et al., Glia (2005) 52:289-300.).

GFAPdelta

Glial Fibrillary Acidic Protein-delta (GFAP-delta). The antibody does not recognize GFAP-alpha protein.

• Western Blotting
• Immunohistochemistry (Paraffin)

Western blot: 1:500 with overnight incubation. The antibody recognizes the ~60 kDa GFAP-delta protein.

Immunohistochemistry: 1:500 incubated for 36-48 hours at 2-8°C on paraffin embedded tissue sections. It is suggested that the tissue be treated with microwave antigen retrieval prior to staining.

Optimal working dilutions must be determined by the end user.

Human

Synthetic peptide from human GFAP-delta.

Rabbit serum. Liquid.

Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.

• GFAP
• FLJ45472

Neuroscience

Neuronal & Glial Markers

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Chemicon (Millipore)

Serum

Rabbit

Polyclonal Antibody