LMX1B anticorps (AA 111-210) (Biotin)

N° du produit ABIN1393279

Détail du produit anti-LMX1B anticorps (Biotin)

Antigène: LMX1B (LIM Homeobox Transcription Factor 1, beta (LMX1B))

Épitope: AA 111-210

Reactivité: Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp LMX1B est conjugé à/à la Biotin

Application: Western Blotting (WB), ELISA, Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))

Réactivité croisée: Souris, Rat

Homologie: Human,Dog,Cow,Sheep,Pig

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human LMX1b/NPS1

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000
IHC-P 1:200-400
IHC-F 1:100-500

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C for 12 months.

Date de péremption: 12 months

Détails sur LMX1B

Antigène: LMX1B (LIM Homeobox Transcription Factor 1, beta (LMX1B))

Autre désignation: LMX1b/NPS1 (LMX1B Produits)

Synonymes: anticorps LMX1.2, anticorps NPS1, anticorps LMX, anticorps LMX1, anticorps LMX1.1, anticorps lmx1b, anticorps lmx1b.1, anticorps Xlmx1b, anticorps lmx1b.2, anticorps LIM homeobox transcription factor 1 beta, anticorps LIM homeobox transcription factor 1, beta b, anticorps LIM homeobox transcription factor 1, beta, gene 1 S homeolog, anticorps LIM homeobox transcription factor 1, beta a, anticorps LMX1B, anticorps Lmx1b, anticorps lmx1bb, anticorps lmx1b.1.S, anticorps lmx1ba

Sujet:

Synonyms: LIM homeo box transcription factor 1 beta, LIM homeobox transcription factor 1 beta, LIM homeobox transcription factor 1-beta, LIM-homeobox protein 1.2, LIM/homeobox protein 1.2, LIM/homeobox protein LMX1B, LMX 1.2, LMX-1.2, LMX1.2, LMX1B, LMX1B_HUMAN, NPS 1, NPS1.

Background: Nail-patella syndrome (NPS) is an autosomal dominant disorder characterized by dyplasia of finger nails, skeletal anomalies and, frequently, renal disease. NPS is caused by putative loss-of-function mutations in the transcription factor LMX1B. LMX1B belongs to the LIM-homeodomain family, members of which are known to be important for pattern formation during development. Twenty-two novel mutations may occur in the gene encoding LMX1B and the type and distribution of the mutations support the hypothesis that NPS is the result of haploinsufficiency for LMX1B. LMX1B is also necessary for normal development of the eye and in regulating dopaminergic neurogenesis and may be involved in developmental glaucoma and the aetiology of idiopathic Parkinson?s disease. Specifically, LMX1B along with LIM1 control the initial trajectory of motor axons in the developing mammalian limb. In addition, LMX1B directly regulates the coordinated expression of alpha 3(IV) and alpha 4(IV) collagen required for normal glomerular basement membrane (GBM) morphogenesis, and the dysregulation of LMX1B in GBM contributes to the renal pathology and nephrosis in NPS.

ID gène: 4010

UniProt: O60663

Pathways: Dopaminergic Neurogenesis