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Incontinentia pigmenti: multisistemic genodermatosis

Incontinentia pigmenti: multisistemic genodermatosis
Posted by Anna

Incontinence pigmenti is an X-linked genodermatosis generally lethal in males; thus, present almost exclusively in women. It is caused by mutations of loss-of-function in IKBKG (inhibitor of kappa polypeptide enhancer genes in B cells, kinase gamma) gene that prevents the NFкβ (nuclear factor kappa-light-chain-enhancer of B cells activated) protein from migrating into the core to initiate transcription factor that strengthens the body’s immune response and preventing apoptosis.

As a result, the mutant cells become susceptible to apoptosis when exposed to cytokines and, in turn, causes vaso-occlusion and ischemia tissue, such as skin, central nervous system and retina. dermatological lesions are characteristic and occurred in 100% of patients; they are distributed along the lines of Blaschko, which follows the pattern of migration of skin cells in embryogenesis. Skin manifestations follow a sequence of four phases of birth: vesicular, verrucous, hyper- and hypopigmentation. These lesions relevant to this disease because they guide the doctor to diagnosis. In addition, they are accompanied by neurological disorders, such as seizures, and some manifestations of ophthalmology, such as retinal detachment. Incontinence pigmenti patients uncompromised eye drops or clinically significant neurologic prognosis is good and normal life expectancy. This abnormality is permanent, which could be a cause of concern for the patient.


He Incontinence pigmenti es una genodermatosis ligada al cromosoma X, generalmente lethal en los hombres. Está causada por una mutación con Perdida de funcion en el gen IKBKG (inhibitor of kappa polypeptide enhancer genes in B cells kinase gamma), que impide que la protein NFкβ (nuclear factor kappa-light-chain-enhancer of B cells activated) al Migre Nucleo comience y que la transcripción de factores amplifican y la respuesta inmunitaria previenen la apoptosis.

Por tanto, las células Mutantes vuelven vulnerables se cuando la apoptosis child citocinas y provocan expuestas a vaso-oclusión e isquemia de Tejidos como la piel, el sistema nervioso central y la retina. Las lesiones dermatológicas Características child; distribuyen se lo largo de las Líneas de Blaschko, las cuales siguen El Patron de migración de las células de la piel en la embryogenesis, ocurren y en el 100% de los pacientes. Las manifestaciones cutáneas aparecen en una secuencia de cuatro fases inicia que desde el nacimiento: vesicular, verrucosa, hiperpigmentada e hipopigmentada. Estas son lesiones relevantes, orientan al Puesto que hacia el diagnóstico Clinico.

Además, se acompañan de anomalías neurológicas, como convulsivas crisis, oftalmológicas manifestaciones multiples y, como el desprendimiento de la retina. Los pacientes con Incontinence pigmenti, pero sin Compromiso oftalmológico o neurológico clínicamente significativo, Tienen un pronóstico bueno y una esperanza de vida normal. Las anomalías que se presentan permanentes child, lo que puede generar preocupación en los pacientes.

Incontinentia pigmenti: multisistemic genodermatosis
Incontinentia pigmenti: multisistemic genodermatosis

Anti-inflammatory effects of Lasia spinosa Leaf Extract on Lipopolysaccharide-Induced RAW 264.7 macrophages.

Lasia spinosa (L.) Thwaites used as a traditional medicine to treat many inflammatory diseases for centuries. However, its effects on the inflammatory response has not been marked. In this study, we investigated the anti-inflammatory activity of spinosa L. leaf extract in lipopolysaccharide (LPS) -induced RAW 264.7 macrophages. We found that the ethanol extract of the leaves spinosa L. showed anti-oxidant activity because of their high levels of polyphenolic compounds.

Phospho-IκBα-S32 Rabbit mAb

AP0707-20ul 20 ul
EUR 221

Phospho-IκBα-S32 Rabbit mAb

AP0707-50ul 50 ul
EUR 308

Anti-Phospho-NFKBIA-(S32/36) antibody

STJ29380 100 µl
EUR 393
Description: This gene encodes a member of the NF-kappa-B inhibitor family, which contain multiple ankrin repeat domains. The encoded protein interacts with REL dimers to inhibit NF-kappa-B/REL complexes which are involved in inflammatory responses. The encoded protein moves between the cytoplasm and the nucleus via a nuclear localization signal and CRM1-mediated nuclear export. Mutations in this gene have been found in ectodermal dysplasia anhidrotic with T-cell immunodeficiency autosomal dominant disease.

Polyclonal Phospho-BAD(S32) Antibody

APR09137G 0.1ml
EUR 484
Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human Phospho-BAD(S32) . This antibody is tested and proven to work in the following applications:

Phospho-NFKBIA-S32 Rabbit pAb

AP0731-100ul 100 ul
EUR 384

Phospho-NFKBIA-S32 Rabbit pAb

AP0731-200ul 200 ul
EUR 554

Phospho-NFKBIA-S32 Rabbit pAb

AP0731-20ul 20 ul
EUR 183

Phospho-NFKBIA-S32 Rabbit pAb

AP0731-50ul 50 ul
EUR 265

Phospho-FOS (S32) Antibody

1-CSB-PA000655
  • EUR 222.00
  • EUR 195.00
  • 100ug
  • 50ug
Description: A polyclonal antibody against Phospho-FOS (S32). Recognizes Phospho-FOS (S32) from Human, Mouse, Rat. This antibody is Unconjugated. Tested in the following application: WB, ELISA;WB:1/500-1/2000.ELISA:1/5000

Phospho-Histone H3.3-S32 Rabbit pAb

AP0486-100ul 100 ul
EUR 384

Phospho-Histone H3.3-S32 Rabbit pAb

AP0486-200ul 200 ul
EUR 554

Phospho-Histone H3.3-S32 Rabbit pAb

AP0486-20ul 20 ul
EUR 183

Phospho-Histone H3.3-S32 Rabbit pAb

AP0486-50ul 50 ul
EUR 265

Anti-Phospho-H3F3A-(S32) antibody

STJ29330 100 µl
EUR 393
Description: Histones are basic nuclear proteins that are responsible for the nucleosome structure of the chromosomal fiber in eukaryotes. Two molecules of each of the four core histones (H2A, H2B, H3, and H4) form an octamer, around which approximately 146 bp of DNA is wrapped in repeating units, called nucleosomes. The linker histone, H1, interacts with linker DNA between nucleosomes and functions in the compaction of chromatin into higher order structures. This gene contains introns and its mRNA is polyadenylated, unlike most histone genes. The protein encoded is a replication-independent member of the histone H3 family.

IKB alpha(Phospho-S32) Antibody

13376-100ul 100ul
EUR 333

IKB alpha(Phospho-S32) Antibody

13376-50ul 50ul
EUR 239

Anti-Phospho-NFKBIA-(S32) antibody

STJ116373 100 µl
EUR 393
Description: This gene encodes a member of the NF-kappa-B inhibitor family, which contain multiple ankrin repeat domains. The encoded protein interacts with REL dimers to inhibit NF-kappa-B/REL complexes which are involved in inflammatory responses. The encoded protein moves between the cytoplasm and the nucleus via a nuclear localization signal and CRM1-mediated nuclear export. Mutations in this gene have been found in ectodermal dysplasia anhidrotic with T-cell immunodeficiency autosomal dominant disease.

Phospho-NFKBIA (S32/S36) Antibody

1-CSB-PA000559
  • EUR 222.00
  • EUR 195.00
  • 100ug
  • 50ug
Description: A polyclonal antibody against Phospho-NFKBIA (S32/S36). Recognizes Phospho-NFKBIA (S32/S36) from Human, Mouse, Rat, Monkey. This antibody is Unconjugated. Tested in the following application: WB, IHC, IF, ELISA;WB:1/500-1/2000.IHC:1/100-1/300.IF:1/200-1/1000.ELISA:1/10000

Anti-Phospho-IKB alpha (S32) Rabbit Monoclonal Antibody

P01139 100ug/vial
EUR 397
Description: Anti-Phospho-IKB alpha (S32) Rabbit Monoclonal Antibody tested for IP, WB in Human

MRP-S32 Polyclonal Antibody

ES5644-50ul 50ul
EUR 207
Description: A Rabbit Polyclonal antibody against MRP-S32 from Human. This antibody is tested and validated for IHC, WB, ELISA

MRP-S32 Polyclonal Antibody

ES5644-100ul 100ul
EUR 279
Description: A Rabbit Polyclonal antibody against MRP-S32 from Human. This antibody is tested and validated for IHC, WB, ELISA

MRP-S32 Polyclonal Antibody

ABP54645-003ml 0.03ml
EUR 158
Description: A polyclonal antibody for detection of MRP-S32 from Human. This MRP-S32 antibody is for IHC-P, ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from the Internal region of human MRP-S32 at AA rangle: 50-130

MRP-S32 Polyclonal Antibody

ABP54645-01ml 0.1ml
EUR 289
Description: A polyclonal antibody for detection of MRP-S32 from Human. This MRP-S32 antibody is for IHC-P, ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from the Internal region of human MRP-S32 at AA rangle: 50-130

MRP-S32 Polyclonal Antibody

ABP54645-02ml 0.2ml
EUR 414
Description: A polyclonal antibody for detection of MRP-S32 from Human. This MRP-S32 antibody is for IHC-P, ELISA. It is affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogenand is unconjugated. The antibody is produced in rabbit by using as an immunogen synthesized peptide derived from the Internal region of human MRP-S32 at AA rangle: 50-130

Anti-Phospho-c-Fos (S32) antibody

STJ90602 200 µl
EUR 197
Description: Rabbit polyclonal to Phospho-c-Fos (S32).

IKB alpha(Phospho-S32) Conjugated Antibody

C13376 100ul
EUR 397

Anti-Phospho- kappa-alpha (S32/S36) antibody

STJ90311 200 µl
EUR 197
Description: IkappaB‚Äêalpha is a protein encoded by the NFKBIA gene which is approximately 35,6 kDa. IkappaB‚Äêalpha is localised to the cytoplasm and nucleus. It is involved in activated TLR4 signalling, 4-1BB pathway and toll-like receptor signalling pathways. It inhibits the activity of dimeric NF-kappa-B/REL complexes by trapping REL dimers in the cytoplasm through masking of their nuclear localization signals. IkappaB‚Äêalpha is expressed in adherent monocytes. Mutations in the NFKBIA gene may result in ectodermal dysplasia with T-cell immunodeficiency. STJ90311 is a synthesized peptide derived from human IkappaB-alpha around the phosphorylation site of S32/S36. This primary antibody specifically binds to endogenous IkappaB‚Äêalpha protein which only binds about S23/36 when S23/36 is phosphorylated.

HSV-?(Herpes Simplex Virus ?Antibody IgG) ELISA test

36 96T/Box Ask for price
Description: ELISA based test for quantitative detection of HSV-?(Herpes Simplex Virus ?Antibody IgG)

Cardboard freezer box, hinged lid, insert for 36 screw-cap 5ml tubes, 5.25 x 5.25 x 3 inches

C2580-36 5/pack
EUR 63.37

Anti-MRP-S32 antibody

STJ94251 200 µl
EUR 197
Description: Rabbit polyclonal to MRP-S32.

Polyclonal BMP3 Antibody (aa23-36)

APR15151G 0.05mg
EUR 484
Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human BMP3 (aa23-36). This antibody is tested and proven to work in the following applications:

HIST1H3A (Ab-36) Polyclonal Antibody

A69371 100 µl
EUR 628.55
Description: kits suitable for this type of research

Rabbit Anti-Human STMN1 (aa.36-40) Polyclonal Antibody

CPB-1108RH 102ul
EUR 460

Rabbit Anti-Human PPP1R14A (aa 36-40) Polyclonal Antibody

CPB-1163RH 100 ul
EUR 460

Rabbit interleukin 36 alpha

ERTI0397 96Tests
EUR 521

PRC1(Phospho-Thr481)Rabbit Polyclonal Antibody

ES8619-100ul 100ul
EUR 279
Description: A Rabbit Polyclonal antibody against PRC1(Phospho-Thr481)Rabbit from Human. This antibody is tested and validated for WB, ELISA, WB, ELISA

PRC1(Phospho-Thr481)Rabbit Polyclonal Antibody

ES8619-50ul 50ul
EUR 207
Description: A Rabbit Polyclonal antibody against PRC1(Phospho-Thr481)Rabbit from Human. This antibody is tested and validated for WB, ELISA, WB, ELISA

ATM Phospho (Ser1981) Rabbit Polyclonal Antibody

Ab11236-050 50ul
EUR 347

ATM Phospho (Ser1981) Rabbit Polyclonal Antibody

Ab11236-100 100ul
EUR 482

Polyclonal Connexin 36 Antibody (C-term)

APR15548G 0.1ml
EUR 484
Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human Connexin 36 (C-term). This antibody is tested and proven to work in the following applications:

Rabbit Anti-Rabbit PRKCQ Polyclonal Antibody, Phospho-Ser695

CPB-723RH 100 ul
EUR 559

Rabbit Anti-Rabbit CTNNB1 Polyclonal Antibody, Phospho-Ser33

CPB-758RH 100 ul
EUR 559

Rabbit Anti-Rabbit CTNNB1 Polyclonal Antibody, Phospho-Ser37

CPB-759RH 100 ul
EUR 559

Rabbit Anti-Rabbit SYN1 Polyclonal Antibody, Phospho-Ser9

CPB-795RH 100 ul
EUR 559

Rabbit Anti-Rabbit Th Polyclonal Antibody, Phospho-Ser19

CPB-840RR 100 ul
EUR 559

Rabbit Anti-Rabbit PIK3R1 Polyclonal Antibody, Phospho-Tyr467

CPB-847RH 100 ul
EUR 559

Rabbit Anti-Rabbit ERBB3 Polyclonal Antibody, Phospho-Tyr1328

CPB-848RH 100 ul
EUR 559

Rabbit Anti-Rabbit EIF4G1 Polyclonal Antibody, Phospho-Ser1232

CPB-851RH 100 ul
EUR 559

Rabbit Interleukin 36 ELISA kit

E04I0390-192T 192 tests
EUR 1270
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin 36 ELISA kit

E04I0390-48 1 plate of 48 wells
EUR 520
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin 36 ELISA kit

E04I0390-96 1 plate of 96 wells
EUR 685
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Histone H3(Phospho-Thr3) Rabbit Polyclonal Antibody

12083-100ul 100ul
EUR 252

Histone H3(Phospho-Ser10) Rabbit Polyclonal Antibody

12084-100ul 100ul
EUR 252

Histone H3(Phospho-Thr11) Rabbit Polyclonal Antibody

12085-100ul 100ul
EUR 252

Histone H3(Phospho-Ser28) Rabbit Polyclonal Antibody

12086-100ul 100ul
EUR 252

Histone H3(Phospho-Thr32) Rabbit Polyclonal Antibody

12087-100ul 100ul
EUR 252

Histone H3(Phospho-Tyr41) Rabbit Polyclonal Antibody

12088-100ul 100ul
EUR 252

Histone H3(Phospho-Thr45) Rabbit Polyclonal Antibody

12089-100ul 100ul
EUR 252

Histone H3(Phospho-Thr118) Rabbit Polyclonal Antibody

12090-100ul 100ul
EUR 252

Histone H4(Phospho-Ser1) Rabbit Polyclonal Antibody

12091-100ul 100ul
EUR 252

Histone H4(Phospho-Ser47) Rabbit Polyclonal Antibody

12092-100ul 100ul
EUR 252

Histone H4(Phospho-Thr80) Rabbit Polyclonal Antibody

12093-100ul 100ul
EUR 252

Histone H2B(Phospho-Ser32) Rabbit Polyclonal Antibody

12094-100ul 100ul
EUR 252

Histone H2A(Phospho-Ser129) Rabbit Polyclonal Antibody

12095-100ul 100ul
EUR 252

Histone H1(Phospho-Ser1) Rabbit Polyclonal Antibody

12096-100ul 100ul
EUR 252

Histone H1(Phospho-Thr3) Rabbit Polyclonal Antibody

12097-100ul 100ul
EUR 252

Histone H2A.X(Phospho-Thr120) Rabbit Polyclonal Antibody

12099-100ul 100ul
EUR 252

Histone H2A.X(Phospho-Ser139) Rabbit Polyclonal Antibody

12100-100ul 100ul
EUR 252

Histone H2A.X(Phospho-Tyr142) Rabbit Polyclonal Antibody

12101-100ul 100ul
EUR 252

Histone H2B(Phospho-Ser14) Rabbit Polyclonal Antibody

12102-100ul 100ul
EUR 252

Polyclonal Neuropeptide Y / NPY Antibody (aa31-36)

APR08720G 0.05ml
EUR 484
Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human Neuropeptide Y / NPY (aa31-36). This antibody is tested and proven to work in the following applications:

Polyclonal PPARA / PPAR Alpha Antibody (aa22-36)

AMR09447G 1 ea
EUR 484
Description: A polyclonal antibody raised in Rabbit that recognizes and binds to Human PPARA / PPAR Alpha (aa22-36). This antibody is tested and proven to work in the following applications:

Histone H3(Phospho-Thr3) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12083 100ul
EUR 397

Histone H3(Phospho-Ser10) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12084 100ul
EUR 397

Histone H3(Phospho-Thr11) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12085 100ul
EUR 397

Histone H3(Phospho-Ser28) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12086 100ul
EUR 397

Histone H4(Phospho-Ser1) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12091 100ul
EUR 397

Histone H2A.X(Phospho-Tyr142) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12101 100ul
EUR 397

Histone H2B(Phospho-Ser14) Rabbit Polyclonal Polyclonal Conjugated Antibody

C12102 100ul
EUR 397

Rabbit Anti-CNP monoclonal antibody, clone KG21-36

DCABH-6204 100 ul
EUR 777

Rabbit Anti-EIF4EBP1 monoclonal antibody, clone KK10-36

DCABH-9646 100 ul
EUR 777

Rabbit Anti-LSP1 monoclonal antibody, clone TO17-36

CABT-L765 100 ul
EUR 777

Rabbit Apelin 36 (AP36) ELISA Kit

abx362263-96tests 96 tests
EUR 754

Rabbit apelin 36(AP36) ELISA kit

E04A1567-192T 192 tests
EUR 1270
Description: A competitive ELISA for quantitative measurement of Rabbit apelin 36(AP36) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit apelin 36(AP36) ELISA kit

E04A1567-48 1 plate of 48 wells
EUR 520
Description: A competitive ELISA for quantitative measurement of Rabbit apelin 36(AP36) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit apelin 36(AP36) ELISA kit

E04A1567-96 1 plate of 96 wells
EUR 685
Description: A competitive ELISA for quantitative measurement of Rabbit apelin 36(AP36) in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin 36 β ELISA kit

E04I0119-192T 192 tests
EUR 1270
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 β in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin 36 β ELISA kit

E04I0119-48 1 plate of 48 wells
EUR 520
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 β in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin 36 β ELISA kit

E04I0119-96 1 plate of 96 wells
EUR 685
Description: A competitive ELISA for quantitative measurement of Rabbit Interleukin 36 β in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin-36 alpha ELISA kit

E04I0767-192T 192 tests
EUR 1270
Description: A sandwich ELISA for quantitative measurement of Rabbit Interleukin-36 alpha in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin-36 alpha ELISA kit

E04I0767-48 1 plate of 48 wells
EUR 520
Description: A sandwich ELISA for quantitative measurement of Rabbit Interleukin-36 alpha in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Rabbit Interleukin-36 alpha ELISA kit

E04I0767-96 1 plate of 96 wells
EUR 685
Description: A sandwich ELISA for quantitative measurement of Rabbit Interleukin-36 alpha in samples from blood, plasma, serum, cell culture supernatant and other biological fluids. This is a high quality ELISA kit developped for optimal performance with samples from the particular species.

Mouse Interleukin-36 alpha (IL-36 alpha) Antibody

32103-05111 150 ug
EUR 261

Rabbit Anti-Human Histone H2A.x (phospho S139) polyclonal Rabbit Antibody

CABT-BL6118 100 ul
EUR 710

Treatment with leaf extract significantly suppressed the production of inflammatory mediators such as nitric oxide and reactive oxygen species and pro-inflammatory cytokine expression in LPS-stimulated RAW 264.7 cells.

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