Metabolism Antibodies 1

Anti-INVS Antibody (CAB10298)

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SKU:
CAB10298
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Metabolism

Description

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Antibody Name:Anti-INVS Antibody
Antibody SKU:CAB10298
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-90 of human INVS (NP_055240.2).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:293T, K-562, Mouse liver, Mouse kidney
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 1-90 of human INVS (NP_055240.2).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:MNKS ENLL FAGS SLAS QVHA AAVN GDKG ALQR LIVG NSAL KDKE DQFG RTPL MYCV LADR LDCA DALL KAGA DVNK TDHS QRTA LHLA AQ
Gene ID:27130
Uniprot:Q9Y283
Cellular Location:Cell projection, Cytoplasm, Membrane, Nucleus, Peripheral membrane protein, cilium, cytoskeleton, spindle
Calculated MW:10kDa/99kDa/117kDa
Observed MW:120kDa
Synonyms:INVS, INV, NPH2, NPHP2, inversin
Background:This gene encodes a protein containing multiple ankyrin domains and two IQ calmodulin-binding domains. The encoded protein may function in renal tubular development and function, and in left-right axis determination. This protein interacts with nephrocystin and infers a connection between primary cilia function and left-right axis determination. A similar protein in mice interacts with calmodulin. Mutations in this gene have been associated with nephronophthisis type 2. Multiple transcript variants encoding distinct isoforms have been identified for this gene.
UniProt Protein Function:INVS: Required for normal renal development and establishment of left-right axis. Probably acts as a molecular switch between different Wnt signaling pathways. Inhibits the canonical Wnt pathway by targeting cytoplasmic disheveled (DVL1) for degradation by the ubiquitin-proteasome. This suggests that it is required in renal development to oppose the repression of terminal differentiation of tubular epithelial cells by Wnt signaling. Involved in the organization of apical junctions in kidney cells together with NPHP1, NPHP4 and RPGRIP1L/NPHP8. Does not seem to be strictly required for ciliogenesis. Defects in INVS are the cause of nephronophthisis type 2 (NPHP2); also known as infantile nephronophthisis. NPHP2 is an autosomal recessive disorder resulting in end-stage renal disease. It is characterized by early onset and rapid progression. Phenotypic manifestations include enlarged kidneys, chronic tubulo-interstitial nephritis, anemia, hyperkalemic metabolic acidosis. Some patients also display situs inversus. Pathologically, it differs from later-onset nephronophthisis by the absence of medullary cysts and thickened tubular basement membranes and by the presence of cortical microcysts. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Chromosomal Location of Human Ortholog: 9q31.1

Molecular Function:protein binding

Disease: Nephronophthisis 2

NCBI Summary:This gene encodes a protein containing multiple ankyrin domains and two IQ calmodulin-binding domains. The encoded protein may function in renal tubular development and function, and in left-right axis determination. This protein interacts with nephrocystin and infers a connection between primary cilia function and left-right axis determination. A similar protein in mice interacts with calmodulin. Mutations in this gene have been associated with nephronophthisis type 2. Multiple transcript variants encoding distinct isoforms have been identified for this gene. [provided by RefSeq, May 2012]
UniProt Code:Q9Y283
NCBI GenInfo Identifier:68565551
NCBI Gene ID:27130
NCBI Accession:Q9Y283.2
UniProt Secondary Accession:Q9Y283,Q2NKL0, Q5W0T6, Q8IVX8, Q9BRB9, Q9Y488, Q9Y498 A2A2Y2,
UniProt Related Accession:Q9Y283
Molecular Weight:118kDa
NCBI Full Name:Inversin
NCBI Synonym Full Names:inversin
NCBI Official Symbol:INVS  
NCBI Official Synonym Symbols:INV; NPH2; NPHP2  
NCBI Protein Information:inversin
UniProt Protein Name:Inversin
UniProt Synonym Protein Names:Inversion of embryo turning homolog; Nephrocystin-2
Protein Family:Inversin
UniProt Gene Name:INVS  
Anti-INVS Antibody (CAB10298)Western blot analysis of extracts of various cell lines, using INVS antibody (CAB10298) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s.
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