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  • 产品名称:Anti-PAX3 Antibody

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简单介绍:
Anti-PAX3 Antibody
详情介绍:
Name: Anti-PAX3 Antibody
See all PAX3 primary antibodies
Description: Goat polyclonal antibody to PAX3.
Applications: ELISA, WB, IHC
Reactivity: Human
Immunogen: Synthetic peptide corresponding to Human PAX3 (N terminal).
Sequence: TTLAGAVPRMM-C
Host: Goat
Clonality: Polyclonal
Isotype: IgG
Conjugate: Unconjugated
Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration: 100 μg at 0.5 mg/ml.
Product Form: Liquid
Formulation: Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Function: Transcription factor that may regulate cell proliferation, migration and apoptosis. Involved in neural development and myogenesis.
Involvement in Disease: Waardenburg syndrome 1: WS1 is an autosomal dominant disorder characterized by non-progressive sensorineural deafness, pigmentary disturbances such as frontal white blaze of hair, heterochromia of irides, white eyelashes, leukoderma, and wide bridge of nose owing to lateral displacement of the inner canthus of each eye (dystopia canthorum). WS1 shows variable clinical expression and some affected individuals do not manifest hearing impairment or iris pigmentation disturbances. Dystopia canthorum is the most consistent sign and is found in 98% of the patients.

Waardenburg syndrome 3: WS3 is an autosomal dominant disorder characterized by sensorineural deafness, pigmentary disturbances, dystopia canthorum and limb anomalies such as hypoplasia of the musculoskeletal system, flexion contractures, fusion of the carpal bones, syndactylies.

Craniofacial-deafness-hand syndrome: Thought to be an autosomal dominant disease which comprises absence or hypoplasia of the nasal bones, hypoplastic maxilla, small and short nose with thin nares, limited movement of the wrist, short palpebral fissures, ulnar deviation of the fingers, hypertelorism and profound sensory-neural deafness.

Rhabdomyosarcoma 2: A form of rhabdomyosarcoma, a highly malignant tumor of striated muscle derived from primitive mesenchymal cells and exhibiting differentiation along rhabdomyoblastic lines. Rhabdomyosarcoma is one of the most frequently occurring soft tissue sarcomas and the most common in children. It occurs in four forms: alveolar, pleomorphic, embryonal and botryoidal rhabdomyosarcomas.
Sequence Similarities: Belongs to the paired homeobox family.
Cellular Location: Nucleus.
Database Links:
  • Entrez Gene: 5077?Human
  • Omim: 606597?Human
  • SwissProt: P23760?Human
  • Unigene: 42146?Human
  • Synonyms:
  • CDHS Antibody
  • HUP 2 Antibody
  • HUP2 Antibody
  • MGC120381 Antibody
  • MGC120382 Antibody
  • MGC120383 Antibody
  • MGC120384 Antibody
  • MGC134778 Antibody
  • Paired box 3 Antibody
  • Paired box gene 3 Antibody
  • Paired box homeotic gene 3 Antibody
  • Paired box protein Pax 3 Antibody
  • Paired box protein Pax-3 Antibody
  • Paired box protein Pax3 Antibody
  • Paired domain gene 3 Antibody
  • Paired domain gene HuP2 Antibody
  • PAX 3 Antibody
  • PAX3 Antibody
  • PAX3/FKHR fusion gene Antibody
  • PAX3_HUMAN Antibody
  • Sp Antibody
  • splotch Antibody
  • Waardenburg syndrome 1 Antibody
  • WS 1 Antibody
  • WS1 Antibody
  • WS3 Antibody
  • Information: Target information shown above is from the UniProt Consortium.
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