Peptide

CyclicPepedia Knowledge Base

Visitor
Visitor
Status Profile

Reference

Pubmed_ID Title DOI Journal
12970295 Identification and functional assessment of novel and known insulin receptor mutations in five patients with syndromes of severe insulin resistance 10.1210/jc.2003-030034.

J Clin Endocrinol Metab

Identification and functional assessment of novel and known insulin receptor mutations in five patients with syndromes of severe insulin resistance

Abstract

  • We analyzed the insulin receptor gene in four patients with leprechaunism and one with type A insulin resistance. We detected novel and previously reported mutations. The novel mutants were expressed in Chinese hamster ovary cells to evaluate the consequences for insulin receptor function. A type A insulin resistance patient from Morocco was homozygous for Arg252His mutation, similar to a previously described type A patient from Japan. A patient with leprechaunism was homozygous for the Ser323Leu mutation, previously identified in homozygous form in two patients with Rabson-Mendenhall syndrome. Phenotypic expression of this mutation is variable. A patient with leprechaunism is compound heterozygous for the previously described Arg1092Trp mutation and a nonsense mutation in codon 897. Another patient with leprechaunism was homozygous for a novel Asn431Asp mutation, which only partially reduces insulin proreceptor processing and activation of signaling cascades. The novel Leu93Gln mutation that fully disrupts proreceptor processing was found in one allele in a patient with leprechaunism. A nonsense mutation at codon 1122 was in the other allele. These results expand the number of pathogenic insulin receptor mutations and demonstrate the variability in their phenotypic expression. The biochemical analysis of mutant insulin receptors does not reliably predict whether the phenotype will be leprechaunism, the Rabson-Mendenhall syndrome, or type A insulin resistance. The previously reported correlation between fibroblast insulin binding and duration of patient survival was not observed.
12970353 Structural basis for tetrodotoxin-resistant sodium channel binding by mu-conotoxin SmIIIA 10.1074/jbc.M309222200.

J Biol Chem

Structural basis for tetrodotoxin-resistant sodium channel binding by mu-conotoxin SmIIIA

Abstract

  • SmIIIA is a new micro-conotoxin isolated recently from Conus stercusmuscarum. Although it shares several biochemical characteristics with other micro-conotoxins (the arrangement of cysteine residues and a conserved arginine believed to interact with residues near the channel pore), it has several distinctive features, including the absence of hydroxyproline, and is the first specific antagonist of tetrodotoxin-resistant voltage-gated sodium channels to be characterized. It therefore represents a potentially useful tool to investigate the functional roles of these channels. We have determined the three-dimensional structure of SmIIIA in aqueous solution. Consistent with the absence of hydroxyprolines, SmIIIA adopts a single conformation with all peptide bonds in the trans configuration. The spatial orientations of several conserved Arg and Lys side chains, including Arg14 (using a consensus numbering system), which plays a key role in sodium channel binding, are similar to those in other micro-conotoxins but the N-terminal regions differ, reflecting the trans conformation for the peptide bond preceding residue 8 in SmIIIA, as opposed to the cis conformation in micro-conotoxins GIIIA and GIIIB. Comparison of the surfaces of SmIIIA with other micro-conotoxins suggests that the affinity of SmIIIA for TTX-resistant channels is influenced by the Trp15 side chain, which is unique to SmIIIA. Arg17, which replaces Lys in the other micro-conotoxins, may also be important. Consistent with these inferences from the structure, assays of two chimeras of SmIIIA and PIIIA in which their N- and C-terminal halves were recombined, indicated that residues in the C-terminal half of SmIIIA confer affinity for tetrodotoxin-resistant sodium channels in the cell bodies of frog sympathetic neurons. SmIIIA and the chimera possessing the C-terminal half of SmIIIA also inhibit tetrodotoxin-resistant sodium channels in the postganglionic axons of sympathetic neurons, as indicated by their inhibition of C-neuron compound action potentials that persist in the presence of tetrodotoxin.
13610841 Arginine-vasotocin, a synthetic analogue of the posterior pituitary hormones containing the ring of oxytocin and the side chain of vasopressin

None

J Biol Chem

Arginine-vasotocin, a synthetic analogue of the posterior pituitary hormones containing the ring of oxytocin and the side chain of vasopressin

Abstract

  • No profile to view
13730351 Isolation and identification of etiocholan-3 alpha-ol-17-one and etiocholane-3 alpha, 11 beta-diol-17-one from human blood plasma 10.1016/0003-9861(61)90229-6.

Arch Biochem Biophys

Isolation and identification of etiocholan-3 alpha-ol-17-one and etiocholane-3 alpha, 11 beta-diol-17-one from human blood plasma

Abstract

  • No profile to view
13797694 [Ester sulfates of steroid hormones. Isolation and characterization in urines of the ester sulfate of 5 beta-androstane 3 alpha-ol-17-one after administration of 5 beta-androstane 3 alpha-ol 17 one (etiocholanolone) to a child]

None

C R Hebd Seances Acad Sci

[Ester sulfates of steroid hormones. Isolation and characterization in urines of the ester sulfate of 5 beta-androstane 3 alpha-ol-17-one after administration of 5 beta-androstane 3 alpha-ol 17 one (etiocholanolone) to a child]

Abstract

  • No profile to view
13824395 Enteric excretion of metabolites of steroid hormones in the human. II. Isolation of delta 5-androsten-3 beta-ol-17-one from meconium

None

J Biol Chem

Enteric excretion of metabolites of steroid hormones in the human. II. Isolation of delta 5-androsten-3 beta-ol-17-one from meconium

Abstract

  • No profile to view
13859141 [Isolation of a new neurohypophysial hormone, isotocin, present in bony fish] 10.1016/0006-3002(62)90085-9.

Biochim Biophys Acta

[Isolation of a new neurohypophysial hormone, isotocin, present in bony fish]

Abstract

  • No profile to view
13957796 The synthesis of Ser4-Ile8-oxytocin (isotocin) 10.1016/0006-3002(63)91249-6.

Biochim Biophys Acta

The synthesis of Ser4-Ile8-oxytocin (isotocin)

Abstract

  • No profile to view
13961378 Colistimethate sodium: a clinical study 10.1016/S0022-5347(17)64542-7.

J Urol

Colistimethate sodium: a clinical study

Abstract

  • No profile to view
14110448 X-RAY DIFFRACTION OF ACTINOMYCIN C3 10.1038/201261a0.

Nature

X-RAY DIFFRACTION OF ACTINOMYCIN C3

Abstract

  • No profile to view
14161210 X-RAY DIFFRACTION OF ACTINOMYCIN C3 10.1038/201814b0.

Nature

X-RAY DIFFRACTION OF ACTINOMYCIN C3

Abstract

  • No profile to view
14222268 STRUCTURES OF POLYMYXIN B2 AND POLYMYXIN E1 10.1038/204185a0.

Nature

STRUCTURES OF POLYMYXIN B2 AND POLYMYXIN E1

Abstract

  • No profile to view
14248718 STRUCTURES OF POLYMYXIN B2 AND POLYMYXIN E1 10.1038/204993a0.

Nature

STRUCTURES OF POLYMYXIN B2 AND POLYMYXIN E1

Abstract

  • No profile to view
14404760 Arginine vasotocin 10.1038/1841465a0.

Nature

Arginine vasotocin

Abstract

  • No profile to view
14419321 Biosynthesis of valinomycin 10.1139/m60-005.

Can J Microbiol

Biosynthesis of valinomycin

Abstract

  • No profile to view