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Cyclohexanemethyl+isothiocyanate
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Cyclohexanemethyl+isothiocyanate
Catalog Number:
(10349-538)
Supplier:
Bioss
Description:
Defects in PLN are the cause of cardiomyopathy dilated type 1P (CMD1P) . Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death.Defects in PLN are the cause of cardiomyopathy familial hypertrophic type 18 (CMH18) . CMH18 is a hereditary heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The symptoms include dyspnea, syncope, collapse, palpitations, and chest pain. They can be readily provoked by exercise. The disorder has inter- and intrafamilial variability ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death.
Catalog Number:
(10351-968)
Supplier:
Bioss
Description:
Immunoglobulin-like cell surface receptor for CD47. Acts as docking protein and induces translocation of PTPN6, PTPN11 and other binding partners from the cytosol to the plasma membrane. Supports adhesion of cerebellar neurons, neurite outgrowth and glial cell attachment. May play a key role in intracellular signaling during synaptogenesis and in synaptic function (By similarity). Involved in the negative regulation of receptor tyrosine kinase-coupled cellular responses induced by cell adhesion, growth factors or insulin. Mediates negative regulation of phagocytosis, mast cell activation and dendritic cell activation. CD47 binding prevents maturation of immature dendritic cells and inhibits cytokine production by mature dendritic cells.
Catalog Number:
(10485-322)
Supplier:
Bioss
Description:
FBXO36 is a 188 amino acid protein that contains one forty amino acid F-box region, making it a member of the F-box family. F-box proteins are critical components of the SCF (Skp1-CUL-1-F-box protein) type E3 ubiquitin ligase complex and are involved in substrate recognition and recruitment for ubiquitination. F-box proteins are members of a large family that regulates cell cycle, immune response, signaling cascades and developmental programs by targeting proteins, such as cyclins, cyclin-dependent kinase inhibitors, IkB-å and ∫-catenin, for degradation by the proteasome after ubiquitination. Functioning as a component of the SCF complex, FBXO36 is thought to recognize and bind to select phosphorylated proteins, thereby promoting their ubiquitination and subsequent degradation.
Catalog Number:
(10434-066)
Supplier:
Bioss
Description:
EMP-2 is a 167 amino acid multi-pass membrane protein that contains four-transmembrane domains and belongs to the GAS3/PMP22 (growth arrest-specific-3/peripheral myelin protein-22) family. Localized to lipid raft domains in the plasma membrane, EMP-2 regulates the expression of several target proteins and is necessary for blastocyst implantation in the uterine endometrium. Specifically, EMP-2 mediates blastocyst implantation by controlling the cell membrane expression of MHC and glycosylphosphatidylinositol-anchored proteins, as well as Integrins and caveolin-1. In adult tissues, EMP-2 is expressed in heart, lung, ovary and intestine, while fetal expression is highest in kidney, brain and liver. Overexpression of EMP-2 is associated with endometrial adenocarcinoma, suggesting a possible role for EMP-2 in tumorigenesis.
Catalog Number:
(10402-416)
Supplier:
Bioss
Description:
ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters. Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein.
Catalog Number:
(10418-494)
Supplier:
Bioss
Description:
The protein encoded by this gene is a member of the protein tyrosine phosphatase (PTP) family. PTPs are known to be signaling molecules that regulate a variety of cellular processes including cell growth, differentiation, mitotic cycle, and oncogenic transformation. This gene is preferentially expressed in a variety of hematopoietic cells, and is an early response gene in lymphokine stimulated cells. The non-catalytic N-terminus of this PTP can interact with MAP kinases and suppress the MAP kinase activities. This PTP was shown to be involved in the regulation of T cell antigen receptor (TCR) signaling, which was thought to function through dephosphorylating the molecules related to MAP kinase pathway. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq].
Catalog Number:
(10478-218)
Supplier:
Bioss
Description:
GTP-binding proteins (G-proteins)are a family of heterotrimeric proteins that play a critical role in signal transduction by coupling cell surface, 7-transmembrane domain receptors to intracellular signaling pathways including second messenger generation (such as cyclic AMP, calcium and diacylglycerol), protein phosphorylation, ion channel activation, gene induction, cell growth and differentiation. Receptor activation catalyzes the exchange of GTP for GDP bound to the inactive G protein alpha subunit resulting in a conformational change and dissociation of the complex. The G protein alpha and beta-gamma subunits are capable of regulating various cellular effectors. Activation is terminated by a GTPase intrinsic to the G-alpha subunit.
Catalog Number:
(10667-596)
Supplier:
Bioss
Description:
Essential component of the COP9 signalosome complex (CSN), a complex involved in various cellular and developmental processes. The CSN complex is an essential regulator of the ubiquitin (Ubl) conjugation pathway by mediating the deneddylation of the cullin subunits of SCF-type E3 ligase complexes, leading to decrease the Ubl ligase activity of SCF-type complexes such as SCF, CSA or DDB2. The complex is also involved in phosphorylation of p53/TP53, c-jun/JUN, IkappaBalpha/NFKBIA, ITPK1 and IRF8/ICSBP, possibly via its association with CK2 and PKD kinases. CSN-dependent phosphorylation of TP53 and JUN promotes and protects degradation by the Ubl system, respectively. Suppresses G-protein- and mitogen-activated protein kinase-mediated signal transduction.
Catalog Number:
(10484-678)
Supplier:
Bioss
Description:
The protein encoded by this gene is a multifunctional, nuclear phosphoprotein that plays a role in cell cycle progression, apoptosis and cellular transformation. It functions as a transcription factor that regulates transcription of specific target genes. Mutations, overexpression, rearrangement and translocation of this gene have been associated with a variety of hematopoietic tumors, leukemias and lymphomas, including Burkitt lymphoma. There is evidence to show that alternative translation initiations from an upstream, in-frame non-AUG (CUG) and a downstream AUG start site result in the production of two isoforms with distinct N-termini. The synthesis of non-AUG initiated protein is suppressed in Burkitt's lymphomas, suggesting its importance in the normal function of this gene. [provided by RefSeq, Jul 2008].
Catalog Number:
(10476-040)
Supplier:
Bioss
Description:
Part of pre- and post-splicing multiprotein mRNP complexes. Involved in numerous pre-mRNA processing events. Promotes constitutive and exonic splicing enhancer (ESE)-dependent splicing activation by bridging together sequence-specific (SR family proteins, SFRS4, SFRS5 and TRA2B/SFRS10) and basal snRNP (SNRP70 and SNRPA1) factors of the spliceosome. Stimulates mRNA 3'-end cleavage independently of the formation of an exon junction complex. Binds both pre-mRNA and spliced mRNA 20-25 nt upstream of exon-exon junctions. Binds RNA and DNA with low sequence specificity and has similar preference for either double- or single-stranded nucleic acid substrates.
Catalog Number:
(10666-056)
Supplier:
Bioss
Description:
PDZK7, also known as PDZD7, is a 517 amino acid protein that contains two PDZ (DHR) domains. Encoded by a gene that maps to human chromosome 10q24.31, PDZK7 is conserved in dog, mouse and rat, and exists as three alternatively spliced isoforms. PDZK7 is known to interact with Harmonin, MASS1, USH1G and Usherin. Localizing to nucleus, PDZK7 is expressed in retinal pigment epithelium and inner ear. Biallelic inactivation of PDZK7 can cause non-syndromic hearing impairment and chromosomal aberrations, which are linked to non-syndromic sensorineural deafness. PDZK7 mutations are also linked to Usher syndrome, which is characterized by retinitis pigmentosa and sensorineural deafness, and Alzheimer disease. The gene that encodes PDZK7 maps to human chromosome 10q24.31.
Catalog Number:
(10671-460)
Supplier:
Bioss
Description:
IQCJ (IQ domain-containing protein J) is a 159 amino acid protein that contains one IQ domain. Existing as four alternatively spliced isoforms, the gene encoding IQCJ maps to human chromosome 3q25.32. Chromosome 3 houses over 1,100 genes, including a chemokine receptor (CKR) gene cluster and a variety of human cancer-related gene loci. Key tumor suppressing genes on chromosome 3 include those that encode the apoptosis mediator RASSF1, the cell migration regulator HYAL1 and the angiogenesis suppressor SEMA3B. Marfan Syndrome, porphyria, von Hippel-Lindau syndrome, osteogenesis imperfecta and Charcot-Marie-Tooth Disease are a few of the numerous genetic diseases associated with chromosome 3.
Catalog Number:
(10264-294)
Supplier:
Bioss
Description:
Carbonic anhydrases (CAs) are members of a large family of zinc metalloenzymes responsible for catalyzing the reversible hydration of carbon dioxide. CAs show extensive diversity in their distribution and subcellular localization. They are involved in a variety of biological processes, including calcification, bone resorption, respiration, acid-base balance and the formation of aqueous humor, saliva, gastric juice and cerebrospinal fluid. CA X also referred to as Carbonic anhydrase-related protein X (CA-RP X) or Cerebral protein 15, is a member of the carbonic anhydrase family that lacks two of the three Zn-binding motifs essential for carbonic anhydrase activity. For this reason, CA X does not exhibit catalytic activity. It is expressed primarily in brain and kidney and may play a role in brain development.
Catalog Number:
(10242-224)
Supplier:
Bioss
Description:
Angiotensin Converting enzyme is involved in catalyzing the conversion of angiotensin I into a physiologically active peptide angiotensin II. Angiotensin II is a potent vasopressor and aldosterone-stimulating peptide that controls blood pressure and fluid-electrolyte balance. This enzyme plays a key role in the renin-angiotensin system. ACE converts angiotensin I to angiotensin II by release of the terminal His-Leu, this results in an increase of the vasoconstrictor activity of angiotensin. Also able to inactivate bradykinin, a potent vasodilatator. ACE exists in two forms, a 170KD somatic form and a 90KD germinal form. The somatic form is expressed by endothelial cells (especially those of lung capillaries and arterioles), epithelial cells (especially in proximal renal tubules and small intestine), by some neuronal cells and variably by some macrophages and T lymphocytes. The germinal form is expressed by spermatozoa.
Catalog Number:
(10276-816)
Supplier:
Bioss
Description:
Sorting nexin (SNX) proteins are members of a large family of hydrophilic PX (phospholipid-binding motif) domain-containing proteins that interact with a variety of receptor types. SNXs are widely expressed, although the tissue distribution of each SNX mRNA varies. The ability of SNXs to bind specific phospholipids, as well as their tendency to form protein-protein complexes, suggests a role for these proteins in cellular membrane trafficking and protein sorting. SNXs may also function specifically in pro-degradative sorting, internalization, endosomal recycling or simply in endosomal sorting. SNXs partially associate with cellular membranes, despite their hydrophilic nature. SNX7 is unique in that it does not have a coiled coil region like some of the SNX family members. Mutations in the SNX7 gene have not been shown to cause any diseases.
Catalog Number:
(10265-788)
Supplier:
Bioss
Description:
Polycystin-1L3 is a 1,732 amino acid multi-pass membrane protein that contains one PLAT domain, one GPS domain and one C-type lectin domain. Expressed at high levels in placenta and present at lower levels in lung and heart, Polycystin-1L3 is thought to function as an ion-channel regulator that may interact with Polycystin-L and play a role in heteromeric taste channels. The gene encoding Polycystin-1L3 maps to human chromosome 16, which encodes over 900 genes and comprises nearly 3% of the human genome. The GAN gene is located on chromosome 16 and, with mutation, may lead to giant axonal neuropathy, a nervous system disorder characterized by increasing malfunction with growth. The rare disorder Rubinstein-Taybi syndrome is also associated with chromosome 16, as is Crohn's disease, which is a gastrointestinal inflammatory condition.
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