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  • Íÿ¹PEX7¶à¿Ë¡¿¹Ìå

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ÖÐÎÄÃû³Æ :   Íÿ¹PEX7¶à¿Ë¡¿¹Ìå


Ó¢ÎÄÃû³Æ :   Anti-PEX7 rabbit polyclonal antibody


±ð       Ãû :   RD; PBD9B; PTS2R; RCDP1


´¢       ´æ :   Àä¶³£¨-20¡æ£© ±Ü¹â


¿¹       Ô­ :   PEX7

ËÞ      Ö÷ :   Rabbit


·´Ó¦ÖÖÊô :   Human Mouse


Ïà¹ØÀà±ð :   Ò»¿¹


±ê  ¼Ç  Îï :   Unconjugate


¿Ë¡ÀàÐÍ :   rabbit polyclonal


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Background:

This gene encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2). Defects in this gene cause peroxisome biogenesis disorders (PBDs), which are characterized by multiple defects in peroxisome function. There are at least 14 complementation groups for PBDs, with more than one phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene have been associated with PBD complementation group 11 (PBD-CG11) disorders, rhizomelic chondrodysplasia punctata type 1 (RCDP1), and Refsum disease (RD).

Applications:

WB, IHC

Name of antibody:

PEX7

Immunogen:

Synthesized peptide derived from internal of human PEX7.

Full name:

peroxisomal biogenesis factor 7

Synonyms£º

RD; PBD9B; PTS2R; RCDP1

SwissProt:

O00628

IHC positive control:

Human breast carcinoma tissue

IHC Recommend dilution:

50-100

WB Predicted band size:

36 kDa

WB Positive control:

NIH/3T3 cells lysate

WB Recommended dilution:

500-3000







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