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The protein encoded by ABCC6 is a member of the superfamily of ATP-binding cassette (ABC) transporters. Zusätzlich bieten wir Ihnen ABCC6 Kits (18) und ABCC6 Proteine (12) und viele weitere Produktgruppen zu diesem Protein an.
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Human Monoclonal ABCC6 Primary Antibody für ELISA, WB - ABIN559916
Pinckard, Sheehan, Arthur, Schreiber: Constitutive shedding of both p55 and p75 murine TNF receptors in vivo. in Journal of immunology (Baltimore, Md. : 1950) 1997
Show all 2 Pubmed References
ABCC6 knockdown HepG2 cells show: 1) intracellular reductive stress; 2) cell cycle arrest in G1 phase; 3) upregulation of p21Cip p53 (zeige TP53 Antikörper) independent; and 4) downregulation of lamin A/C (zeige LMNA Antikörper). the absence of ABCC6 profoundly changes the HepG2 phenotype, suggesting that Pseudoxanthoma elasticum syndrome is a complex metabolic disease that is not exclusively related to the absence of pyrophosphate in the bloodstream.
ABCC6 deficiency can be rescued by 4-phenylbutyrate therapy in a mouse model expressing human variants
Biochemical and cell biological analyses demonstrate these mutations influence multiple steps in the biosynthetic pathway, minimally altering local domain structure but adversely impacting ABCC6 assembly and trafficking. The differential impacts on local and global protein structure are consistent with hierarchical folding and assembly of ABCC6.
The results suggest that a transmembrane domain is not required for transport function and that a cytosolic loop maintains ABCC6 in a targeting-competent state for the basolateral membrane and might be involved in regulating the nucleotide binding domains.
The results of this study showed that mtDNA(atp6 (zeige MT-ATP6 Antikörper)) variants were actively involved in schizophrenia in some families with maternal inheritance of this
Pseudoxanthoma elasticum is due to mutation of the ABCC6 gene on chromosome 16.
Membrane insertion and topology of the amino-terminal domain TMD0 of multidrug-resistance associated protein 6
A direct relationship between reduced ABCC6 levels and the expression of pro-mineralization genes in hepatocytes.
Minimal rescue of the morpholino-induced phenotype was achieved with eight of the nine mutant human ABCC6 mRNAs tested, implying pathogenicity. This study demonstrates that the Chinese PXE population harbors unique ABCC6 mutations.
Virtual screening expands this possibility to explore more compounds that can interact with ABCC6, and may aid in understanding the mechanisms leading to pseudoxanthoma elasticum
abrogated ABCC6 function does cause alterations in the metabolic profile of the liver in accordance with PXE being a metabolic disease originating from liver disturbance
bisphosphonates may be helpful for prevention of mineral deposits in Pseudoxanthoma elasticum and generalized arterial calcification of infancy caused by mutations in the ABCC6 gene.
In a mouse model of CKD, ABCC6 protein expression was decreased in liver and kidney, however mRNA levels were unchanged.
In the Abcc6(-/-) genotype, dermal fibroblasts actively contribute to changes that promote matrix calcification; these cells can be further modulated with time by the calcified environment, contributing to the age-dependent progression.
This study showed that the expression of ABCC6 in liver is an important determinant of calcification in cardiac tissues in response to injuries
Magnesium oxide reduces carotid intima media thickness in Abcc6-/- mouse model for pseudoxanthoma elasticum.
Studied the role of genetic modulation and the role of diet in nephrocalcinosis using two established mouse models of ectopic mineralization, Abcc6(tm1Jfk) and Enpp1 (zeige ENPP1 Antikörper)(asj (zeige ARSJ Antikörper)) mice.
Report lower elasticity and increased myogenic tone without major changes in agonist-dependent contraction in aged Abcc6(-/-) mouse model of pseudoxanthoma elasticum.
the development of cardiac hypertrophy in the 24-month-old Abcc6(-/-) mice suggests that old pseudoxanthoma elasticum patients might be prone to developing late cardiopathy.
ABCC6 is in the basolateral membrane, mediating the sinusoidal efflux of a metabolite from the hepatocytes to systemic circulation.
The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). The encoded protein, a member of the MRP subfamily, is involved in multi-drug resistance. Mutations in this gene cause pseudoxanthoma elasticum. Alternatively spliced transcript variants that encode different proteins have been described for this gene.
ATP-binding cassette sub-family C member 6
, anthracycline resistance-associated protein
, multi-specific organic anion transporter E
, multidrug resistance-associated protein 6
, ATP-binding cassette, sub-family C, member 6
, multidrug resistance-associated protein-6
, ATP-binding cassette, sub-family C (CFTR/MRP), member 6
, MRP-like protein 1
, liver multidrug resistance-associated protein 6