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Anti-WDR19 Antibody

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Anti-WDR19 Antibody


Anti-WDR19 Antibody  的详细介绍
Name: Anti-WDR19 Antibody
See all WDR19 primary antibodies
Description: Rabbit polyclonal antibody to WDR19
Specificity: The antibody detects endogenous levels of total WDR19 protein.
Applications: IHC
Reactivity: Human
Immunogen: Fusion protein of human WDR19
Host: Rabbit
Clonality: Polyclonal
Conjugate: Unconjugated
Purification: Antigen affinity purification.
Concentration: 1.1mg / ml
Formulation: Rabbit IgG in pH7.4 PBS, 0.05% NaN3, 40% Glycerol.
Storage: Store at -20?C
Function: Component of the IFT complex A (IFT-A), a complex required for retrograde ciliary transport (PubMed:20889716). Involved in cilia function and/or assembly (By similarity). Associates with the BBSome complex to mediate ciliary transport (By similarity).
Tissue Specificity: Some isoforms are tissue-specific. Highly expressed in the prostate. Lower expression in the cerebellum, pituitary gland, fetal lung, and pancreas. In normal prostate, expressed in both basal and luminal epithelial cells. No expression detected in fibromuscular stromal cells, endothelial cells, or infiltrating lymphocytes. Uniformed expression in prostate adenocarcinoma cells.
Involvement in Disease: Cranioectodermal dysplasia 4: A disorder primarily characterized by craniofacial, skeletal and ectodermal abnormalities. Clinical features include craniosynostosis, narrow rib cage, short limbs, brachydactyly, hypoplastic and widely spaced teeth, sparse hair, skin laxity and abnormal nails. Nephronophthisis leading to progressive renal failure, hepatic fibrosis, heart defects, and retinitis pigmentosa have also been described.

Short-rib thoracic dysplasia 5 with or without polydactyly: A form of short-rib thoracic dysplasia, a group of autosomal recessive ciliopathies that are characterized by a constricted thoracic cage, short ribs, shortened tubular bones, and a 'trident' appearance of the acetabular roof. Polydactyly is variably present. Non-skeletal involvement can include cleft lip/palate as well as anomalies of major organs such as the brain, eye, heart, kidneys, liver, pancreas, intestines, and genitalia. Some forms of the disease are lethal in the neonatal period due to respiratory insufficiency secondary to a severely restricted thoracic cage, whereas others are compatible with life. Disease spectrum encompasses Ellis-van Creveld syndrome, asphyxiating thoracic dystrophy (Jeune syndrome), Mainzer-Saldino syndrome, and short rib-polydactyly syndrome.

Nephronophthisis 13: An autosomal recessive disorder resulting in end-stage renal disease. It is a progressive tubulo-interstitial kidney disorder histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts.

Senior-Loken syndrome 8: A renal-retinal disorder characterized by progressive wasting of the filtering unit of the kidney (nephronophthisis), with or without medullary cystic renal disease, and progressive eye disease. Typically this disorder becomes apparent during the first year of life.
Cellular Location: Cell projection > Cilium. Cytoplasm > Cytoskeleton > Cilium basal body. Cell projection > Cilium > Photoreceptor outer segment.

Localizes to photoreceptor connecting cilia, to the base of motile cilia in brain ependymal cells and to the base of and along primary cilia in kidney cells.
Database Links:
  • Entrez Gene: 57728?Human
  • Omim: 608151?Human
  • SwissProt: Q8NEZ3?Human
  • Unigene: 438482?Human
    		•
    Synonyms:
  • DYF 2 Antibody
  • DYF2 Antibody
  • FLJ23127 Antibody
  • IFT144 Antibody
  • Intraflagellar transport 144 homolog Antibody
  • KIAA1638 Antibody
  • ORF26 Antibody
  • Oseg6 Antibody
  • PWDMP Antibody
  • WD repeat containing protein 19 Antibody
  • WD repeat domain 19 Antibody
  • WD repeat membrane protein PWDMP Antibody
  • WD repeat-containing protein 19 Antibody
  • WDR19 Antibody
  • WDR19_HUMAN Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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