anti-SPTAN1 (SPTAN1) Antikörper

Zebrafish Spectrin alpha Chain, Brain (SPTAN1) Interaktionspartner

1. AlphaII-spectrin in the axonal cytoskeleton is essential for stabilizing nascent sodium-channel clusters and assembling the mature node of Ranvier.

Mouse (Murine) Spectrin alpha Chain, Brain (SPTAN1) Interaktionspartner

1. Fodrin is a regulatory transporter of gamma-tubulin to the centrosomes for normal progression of mitosis.

2. Mena/VASP and alphaII-Spectrin complexes regulate cytoplasmic actin networks in cardiomyocytes and protect from conduction abnormalities and dilated cardiomyopathy.

3. Degradation of Spna2 by calpain- and/or caspase is not central to the pathogenesis of 1,2-diacetylbenzene (DAB) axonopathy.

4. The present study demonstrates the age-dependent intraneuronal presence of an alphaII-spectrin cleavage fragment in mammalian forebrain which is exacerbated in a transgenic model of AD.

5. alphaII-spectrin deficiency is embryonic lethal, with cardiac, craniofacial and neural tube malformations.

6. Results of this study provide evidence for a role for spectrin in different steps of megakaryocyte development through its participation in the formation of invaginated membranes and in the maintenance of proplatelet structure.

7. When cleaved into a 120-kDa product by apoptotic proteases in salivary gland cells in vitro, alpha-fodrin is a successful pathogenic autoantigen for development of autoimmune exocrinopathy in the Sjogren syndrome model.

8. In heart muscle, alphaII-spectrin is located within the contractile fibres near the Z-disc as well as on the cardiomyocyte plasma membrane.

9. support a critical role for the alphaII-spectrin-SH3i isoform of spectrin in intracellular targeting of connexin 43 to gap junctions

10. AlphaII-spectrin-VASP complexes regulate cortical actin cytoskeleton assembly with implications for vascular permeability.

11. These data suggest that CLN3 is involved in the regulation of plasma membrane fodrin cytoskeleton and consequently, the plasma membrane association of Na(+), K(+) ATPase.

12. the cortical network involved in the sound-induced electromotility of OHCs contains alphaII-betaV spectrin, and not the conventional alphaII-betaII spectrin.

Human Spectrin alpha Chain, Brain (SPTAN1) Interaktionspartner

1. Spectrin may be engaged in regulation of distinct events necessary for the establishment and maturity of the immunological synapse.

2. SPTAN1-related disorders comprise a wide spectrum of neurodevelopmental phenotypes ranging from mild to severe and progressive. Spectrin aggregate formation in fibroblasts with mutations in the alpha/beta heterodimerization domain seems to be associated with a severe neurodegenerative course.

3. Alpha-spectrin is critical for recruitment of non-ubiquitinated FANCD2 to sites of damage, which has an important role in the repair response and interstrand cross-link repair.

4. Studies demonstrate that alpha-IISp plays a critical role in maintaining chromosome stability in cells after DNA interstrand cross-links damage by repairing damage that occurs in both genomic and telomeric DNA.

5. These results suggest that ubiquitin C-terminal hydrolase and alphaII-spectrin breakdown product 145 kDa may be useful in assessing outcome after pediatric traumatic brain injury.

6. Variations in both alpha-spectrin (SPTA1) and beta-spectrin ( SPTB ) were found in a neonate with prolonged jaundice in a family where nine individuals had hereditary elliptocytosis.

7. aggressiveness of MLH1-positive colorectal cancers might be related to SPTAN1.

8. hypomyelination of the cerebral white matter was revealed at the age of 1 year, and a de novo heterozygous mutation in the SPTAN1 gene was confirmed

9. loss of SPTAN1 switches TGF-beta signaling from tumor suppression to tumor promotion by engaging Notch signaling and activating SOX9 in esophageal adenocarcinoma.

10. organization of a spectrin-like cytoskeleton is associated with keratinocyte differentiation, and cytoskeleton disruption is mediated by either PKCdelta(Thr505) phosphorylation associated with phosphorylated adducin or due to reduction of endogenous adducin

11. In-frame mutations in the C-terminus of SPTAN1 cause a core set of manifestations that include severe intellectual disability, generalized epilepsy, and pontocerebellar atrophy.

12. Analysis of alphaII-spectrin breakdown products in cerebrospinal fluid predicts mortality and injury severity in adults following traumatic brain injury.

13. findings suggest that pathological aggregation of alpha/beta spectrin heterodimers and abnormal axon initial segment(AIS) integrity resulting from SPTAN1 mutations were involved in pathogenesis of infantile epilepsy

14. Fanconi anemia proteins may play an important role in maintaining the stability of alphaIISp in the cell by regulating its cleavage by mu-calpain.

15. This protein has been found differentially expressed in the anterior cingulate cortex from patients with schizophrenia

16. SigA binds to epithelial HEp-2 cells as well as being able to induce fodrin degradation in vitro and in situ, further extending its documented role in the pathogenesis of Shigella infections.

17. Review. 120 kDa alpha-fodrin is an important autoantigen in both animal models and Sjogren syndrome patients. Increased caspase cascade activity may be involved in the progression of alpha-fodrin proteolysis and tissue destruction.

18. High affinity and slow overall kinetics of association/dissociation of alpha II-spectrin may suit it well to a role in strengthening cell junctions and providing stable anchorage for transmembrane proteins at points specified by cell-adhesion molecules.

19. Fanconi anemia cell lines deficient in alphaII spectrin express normal levels of alphaII spectrin mRNA.

20. These observations indicate that calpain is activated and reacts with alpha-fodrin as a substrate at the sarcolemma, and plays a key role in modulating sarcolemmal proteins to adapt to the specific conditions in each myopathy.