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Spectrin, alpha, erythrocytic 1

Spectrin is an actin crosslinking and molecular scaffold protein that links the plasma membrane to the actin cytoskeleton, and functions in the determination of cell shape, arrangement of transmembrane proteins, and organization of organelles. It is a tetramer made up of alpha-beta dimers linked in a head-to-head arrangement. This gene is one member of a family of alpha-spectrin genes. The encoded protein is primarily composed of 22 spectrin repeats which are involved in dimer formation. It forms weaker tetramer interactions than non-erythrocytic alpha spectrin, which may increase the plasma membrane elasticity and deformability of red blood cells. Mutations in this gene result in a variety of hereditary red blood cell disorders, including elliptocytosis type 2, pyropoikilocytosis, and spherocytic hemolytic anemia. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: CAN, HAD, ACID, tissue-nonspecific alkaline phosphatase, Interleukin-3
Papers on HPP
Asfotase Alfa: A Review in Paediatric-Onset Hypophosphatasia.
Scott, Auckland, New Zealand. In Drugs, Feb 2016
Hypophosphatasia (HPP) is a rare inheritable disease that results from loss-of-function mutations in the ALPL gene encoding tissue-nonspecific alkaline phosphatase (TNSALP).
Design, synthesis and evaluation of bitopic arylpiperazinephenyl-1,2,4-oxadiazoles as preferential dopamine D3 receptor ligands.
Cheon et al., Kwangju, South Korea. In Bioorg Med Chem, Feb 2016
Specifically, non-conserved Tyr36, EL1 especially non-conserved Thr92 and Gly94, and EL2 Val180, Cys181 and Ser182 of D3R may contribute to D3R specificity over D2R.
Self-Renewal and High Proliferative Colony Forming Capacity of Late-Outgrowth Endothelial Progenitors is Regulated by Cyclin-Dependent Kinase Inhibitors Driven by Notch Signaling.
Khosrotehrani et al., Australia. In Stem Cells, Feb 2016
Only CD34 + ECFC had the capacity to reproduce high proliferative potential (HPP) colonies on replating, whereas CD34- ECFCs formed only small clusters.
A novel amperometric biosensor for ß-triketone herbicides based on hydroxyphenylpyruvate dioxygenase inhibition: A case study for sulcotrione.
Noguer et al., Perpignan, France. In Talanta, Feb 2016
An amperometric biosensor was designed for the determination of sulcotrione, a β-triketone herbicide, based on inhibition of hydroxyphenylpyruvate dioxygenase (HPPD), an enzyme allowing the oxidation of hydroxyphenylpyruvate (HPP) in homogentisic acid (HGA).
Alkaline Phosphatase and Hypophosphatasia.
Whyte et al., Los Angeles, United States. In Calcif Tissue Int, Dec 2015
UNASSIGNED: Hypophosphatasia (HPP) results from ALPL mutations leading to deficient activity of the tissue-non-specific alkaline phosphatase isozyme (TNAP) and thereby extracellular accumulation of inorganic pyrophosphate (PPi), a natural substrate of TNAP and potent inhibitor of mineralization.
Erythroid-Specific Expression of LIN28A Is Sufficient for Robust Gamma-Globin Gene and Protein Expression in Adult Erythroblasts.
Miller et al., Bethesda, United States. In Plos One, 2014
For this purpose, lentiviral transduction vectors were produced with LIN28A expression driven by erythroid-specific gene promoter regions of the human KLF1 or SPTA1 genes.
Neurological Symptoms of Hypophosphatasia.
Taketani, Izumo, Japan. In Subcell Biochem, 2014
Hypophosphatasia (HPP) is a bone metabolic disorder caused by mutations in the liver/bone/kidney alkaline phosphatase gene (ALPL), which encodes tissue-nonspecific alkaline phosphatase (TNAP).
What Can We Learn About the Neural Functions of TNAP from Studies on Other Organs and Tissues?
Millán, Los Angeles, United States. In Subcell Biochem, 2014
To-date, the function of tissue-nonspecific alkaline phosphatase (TNAP) has largely been defined through studies in patients and mice affected by hypophosphatasia (HPP), a rare inborn-error-of-metabolism caused by mutation(s) in the TNAP gene (ALPL).
Molecular Genetics of Hypophosphatasia and Phenotype-Genotype Correlations.
Mornet, Le Chesnay, France. In Subcell Biochem, 2014
Hypophosphatasia (HPP) is due to deficient activity of the tissue-nonspecific isoenzyme of alkaline phosphatase (TNAP).
Clinical Forms and Animal Models of Hypophosphatasia.
Salles, Toulouse, France. In Subcell Biochem, 2014
Hypophosphatasia (HPP) is due to mutations of the tissue non-specific alkaline phosphatase (TNAP) gene expressed in the liver, kidney, and bone.
New bonding modes of carbon and heavier group 14 atoms Si-Pb.
Parameswaran et al., Marburg an der Lahn, Germany. In Chem Soc Rev, 2014
Tetrylones EL2 are divalent E(0) compounds which possess two lone pairs at E. The unique electronic structure of tetrylones (carbones, silylones, germylones, stannylones, plumbylones) clearly distinguishes them from tetrylenes ER2 (carbenes, silylenes, germylenes, stannylenes, plumbylenes) which have electron-sharing bonds R-E-R and only one lone pair at atom E. The different electronic structures of tetrylones and tetrylenes are revealed by charge- and energy decomposition analyses and they become obvious experimentally by a distinctively different chemical reactivity.
Identification of a conserved aggregation-prone intermediate state in the folding pathways of Spc-SH3 amyloidogenic variants.
Shakhnovich et al., Lisbon, Portugal. In J Mol Biol, 2012
These data further suggest that residues 44 and 53, which are key players in the nucleation-condensation mechanism of folding, are also important triggers of the aggregation process.
Enterohaemorrhagic Escherichia coli requires the spectrin cytoskeleton for efficient attachment and pedestal formation on host cells.
Guttman et al., Canada. In Microb Pathog, 2012
Further studies involving siRNA-mediated knockdowns of spectrin, adducin, or p4.1 revealed that those proteins are needed for efficient docking of enterohaemorrhagic Escherichia coli to host cells.
Apparent structural differences at the tetramerization region of erythroid and nonerythroid beta spectrin as discriminated by phage displayed scFvs.
Fung et al., Chicago, United States. In Protein Sci, 2011
Results suggest that it is possible for cellular proteins to differentially associate with the C-termini of different beta-spectrin isoforms to regulate alpha- and beta-spectrin association to form functional spectrin tetramers.
On the association of lipid rafts to the spectrin skeleton in human erythrocytes.
Minetti et al., Pavia, Italy. In Biochim Biophys Acta, 2011
lipid rafts are associated with the spectrin skeleton in human erythrocytes
Glycosylation of erythrocyte spectrin and its modification in visceral leishmaniasis.
Mandal et al., Calcutta, India. In Plos One, 2010
analysis of glycosylation of erythrocyte spectrin and its modification in visceral leishmaniasis
Multiple loci influence erythrocyte phenotypes in the CHARGE Consortium.
Lin et al., Bethesda, United States. In Nat Genet, 2009
Our findings include loci previously associated with these traits (HBS1L-MYB, HFE, TMPRSS6, TFR2, SPTA1) as well as new associations (EPO, TFRC, SH2B3 and 15 other loci).
WECHE: a novel hematopoietic regulatory factor.
Lasky et al., Kumamoto, Japan. In Immunity, 2000
WECHE reduced HPP-CFC production from fetal liver-derived stem cells.
Defects in semiconductors: some fatal, some vital
Haller et al., Berkeley, United States. In Science, 1998
Metastable defects such as DX centers and the EL2-related arsenic antisite are briefly discussed.
Novel mutations in families with unusual and variable disorders of the skeletal muscle sodium channel.
Brown et al., Boston, United States. In Nat Genet, 1992
Mutations in the skeletal muscle sodium channel gene (SCN4A) have been described in paramyotonia congenita (PMC) and hyperkalaemic periodic paralysis (HPP).
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