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3) Spinal Muscular Atrophy - SMN, Gemins and ProfilinII

SMN (Survival of Motor Neurons) is a protein of 294 amino-acids and Mr of 40kDa. Levels of functional protein are greatly reduced in SMA and are related to disease severity. Gemins(2 - 8)form a functiomal complex with SMN. SIP-1 is a protein which interacts directly with SMN and always co-localizes.SIP-1 is also known as Gemin2.

Abbreviations: wb = western blot if = immunolocalization ip = immunoprecipitation hu = human mo = mouse xe = xenopus lævis ch = chick dm = drosophila fi = fish (raja clavata) CRX = cross-reacting proteins p = preparation m = mapping a = applications ¶ = Also available at Developmental Studies Hybridoma Bank, IOWA(http://dshb.biology.uiowa.edu/)




Epitope (aa)


ve (x-ve)


SMN 22 mAbs

MANSMA1* ¶11F3, 345G1aa AYD-AVAwb, if, iphu, mo(weak),(xrb)34
MANSMA2* ¶8F7, 131G1Exon4 aa ASLwb, ifhu, mo(weak),rb34
MANSMA3 ¶8E1G1Exon5 aa PSGP---Pwb, ifhu, mo(weak),(xrb)34
MANSMA4 ¶10A8G1Exon4 aa ASLwb(weak), ifhu, mo(weak)34
MANSMA5 ¶12G6G1Exon4 aa ASLwb(weak), ifhu, mo(weak)34
MANSMA6 ¶4H2G128-91wb, ifhu, mo34
MANSMA7 ¶1B12, 176G128-91wb, ifhu, mo, fi34
MANSMA8 ¶6H3G1Exon4 aa ASLwb, if(weak)hu, mo(weak)34
MANSMA9 ¶9C7G128-91wb, ifhu, mo(weak)34
MANSMA10 ¶8A10G1Exon4 aa ASLwb, ifhu, mo(weak)34
MANSMA11* ¶6G9G1Exon4 aa ASLwb, ifhu, mo(weak)34
MANSMA12* ¶2E6, 208G1aa DDT-LI---Y--Awb, ifhu, mo, fi34
MANSMA13* ¶5E3G128-91wb, ifhu, mo34
MANSMA14* ¶6A6G128-91wb, ifhu, mo34
MANSMA15 ¶6G1G128-91wb(weak), ifhu, mo(weak)34
MANSMA16 ¶8A6G128-91wb, ifhu, mo34
MANSMA17 ¶8B11G128-91wb(weak), ifhu, mo34
MANSMA18 ¶8B12G128-91wb, ifhu, mo34
MANSMA19 ¶7G12G128-91wb, ifhu, mo34
MANSMA20 ¶8C1G128-91wb, ifhu, mo34
MANSMA21 ¶1F1G128-91wb(weak), ifhu, mo, fi34
MANSMA22 ¶8B3G128-91wb, ifhu,mo(weak)34

SIP-1 4 mAbs

MANSIP1A* ¶1G9, 301G1not mappedwb, ifhu, rb, mo (not monkey)34
MANSIP1B ¶3F8, 83G1not mappedwb, ifhu, rb, mo (not monkey)34
MANSIP1C ¶6D1G1not mappedwb, ifhu, rb, mo (not monkey)34
MANSIP1D ¶7C11G1not mappedwb, ifhu, rb, mo (not monkey)34

Gemin4 06 mAbs

GEM4A1A8IgAaa T-W-EDwb, (xif)hu, pig, mo (xfi)42
GEM4B ¶1G4G1not mappedwb, if)hu, pig (xfi,xmo)42
GEM4C ¶9E6G2baa T-W-EDwb, (xif)hu, pig, mo (xfi)42
GEM4D ¶2H8G1not mappedwb, if)hu, pig (xfi,xmo)42
GEM4E7B5G2anot mappedwb, if)hu, pig (xfi,xmo)42
GEM4F2A2G2baa T-W-EDwb, (xif)hu, pig (xfi,xmo)42

Gemin5 18 mAbs

GEM5A ¶1A5G1not mappedwb, (xif)hu (xpig,xfi,xmo)42
GEM5B1B1G2anot mappedwb, (xif)hu, fi, mo (xpig)42
GEM5C ¶1C10G2bnot mappedwb, (xif)hu, pig, mo (xfi)42
GEM5D2A12G2bnot mappedwb, (xif)hu, pig (xfi,xmo)42
GEM5E2B11G2anot mappedwb, (xif)hu, fi (xpig,xmo)42
GEM5F5C3G2anot mappedwb, (xif)hu, mo (xpig,xfi)42
GEM5G ¶6C4G1not mappedwb, (xif)hu, fi (xpig,xmo)42
GEM5H6E2G2bnot mappedwb, (xif)hu, fi (xpig,xmo)42
GEM5I7A1G1not mappedwb, (xif)hu, fi(xpig,xmo)42
GEM5J7F1G1aa RVS-FTwb, (xif)hu (xmo)42
GEM5K8C7G2bnot mappedwb, (xif)hu, fi (xpig,xmo)42
GEM5L ¶8E7G2bnot mappedwb, (xif)hu, fi (xpig,xmo)42
GEM5M ¶1E12, 207G1not mappedwb,ifhu, pig, mo (xfi)42
GEM5N2E6G1not mappedwb,if(weak)hu, pig, mo (xfi)42
GEM5O3E11G1not mappedwb,if(weak)hu, pig, mo (xfi)42
GEM5P3G2G1not mappedwb,if(weak)hu, pig (xfi,xmo)42
GEM5Q4G7G1not mappedwb,if(weak)hu, pig, mo (xfi)42
GEM5R6G5G1not mappedwb,(xif)hu, pig (xfi,xmo)42

GEM6 07 mAbs

GEM6A ¶2C11G2anot mappedwb, ifhu (xpig,xfi,xmo)42
GEM6B ¶4H6, 134G2anot mappedwb, ifhu, pig (xfi,xmo)42
GEM6C5E9G1not mappedwb, (xif)hu,(xpig,xfi,xmo)42
GEM6D ¶6F5G2anot mappedwb, ifhu,pig (xfi,xmo)42
GEM6E8A9G2anot mappedwb, ifhu,(xpig,xfi,xmo)42
GEM6F ¶8D8G2anot mappedwb, ifhu,pig,(xfi,xmo)42
GEM6G3F12G2bnot mappedwb, ifhu (xpig,xfi,xmo)42

GEM7 07 mAbs

GEM7A ¶2A11G1not mappedwb, ifhu (xpig,xfi,xmo)42
GEM7B6A2G1not mappedwb, ifhu (xpig,xfi,xmo)42
GEM7C6A9G1not mappedwb (xif)hu (xpig,xfi,xmo)42
GEM7D ¶7A12G1not mappedwb,if)hu (xpig,xfi,xmo)42
GEM7E8D8G1not mappedwb,if)hu (xpig,xfi,xmo)42
GEM7F ¶8H1G1not mappedwb,if)hu,pig (xfi,xmo)42
GEM7G10C12G1not mappedwb,if)hu (xpig,xfi,xmo)42

Profilin II 5 mAbs

PF2A1 ¶8F8G2a96-105wb, ifhu, rat43
PF2A2 ¶6D1G2a96-105wb, ifhu, rat43
PF2A3 ¶6D10G2anot mappedwb, ifhu, rat43
PF2A45A6G2a96-105wb, ifhu, rat43
PF2A5 ¶6B3G196-105wb, ifhu, rat43